Dr. Joseph Dearani discusses new developments in surgical techniques and medical management for patients with Ebstein’s Anomaly, a congenital heart defect in which the tricuspid valve doesn’t function properly.
Ebstein Anomaly: Update on Surgical Management
Ebstein anomaly is a rare congenital heart malformation with a seemingly infinite range of anatomic variability. In addition, the clinical presentation is also widely variable, ranging from the need for urgent surgical intervention in a symptomatic neonate to an incidental finding in an asymptomatic adult patient on a routine medical examination. It can be challenging for the cardiologist to know when to refer for operation and for the surgeon to develop a consistent method with reproducible and reliable results. Read full article from Cardiovascular Update
Dr. Manish Kohli discusses new developments in the medical management of prostate cancer. A review article appears in the January 2010 issue of Mayo Clinic Proceedings
ABSTRACT
Prostate cancer is a substantial public health burden and a leading cause of cancer—related morbidity and mortality in the United States despite the observation that annual prostate cancer—specific mortality rates have been declining during the previous decade. Although the reasons for this positive development are unclear, a combination of factors may have contributed. This update will review ongoing developments and summarize therapeutic advances in prostate cancer treatment on the basis of the current understanding of prostate cancer biology. Literature for this review was selected in 2009 by searching PubMed for the following keywords: prostatic neoplasms, castration, androgen receptor, hormonal, and chemotherapy. Emphasis is placed on published clinical studies in advanced prostate cancer therapeutics in the past 5 to 10 years. Also included in the review are novel hormonal agents targeting the androgen receptor currently in development for the treatment of advanced prostate cancer.
AUTHORS Manish Kohli, MD and Donald J. Tindall, PhD from Mayo Clinic
Dr. Andrew McKeon discusses a new Mayo Clinic study on Paraneoplastic Neurologic Disorders with a MedPage Today reporter. The study appears online in early release for the Archives of Neurology.
ABSTRACT Objective To evaluate the cancer detection rate of whole-body positron emission tomography–computed tomography (PET-CT) in a paraneoplastic neurologic context.
Design Retrospective medical record review.
Setting Mayo Clinic, Rochester, Minnesota.
Patients Fifty-six consecutive patients with clinically suspected paraneoplastic neurologic disorders who underwent PET-CT after negative standard evaluations, including CT.
Main Outcome Measure Rate of cancer detection.
Results Abnormalities suggestive of cancer were detected using PET-CT in 22 patients (39%); 10 patients (18%) had cancer confirmed histologically. Cancers detected (limited stage in 9 of 10 patients and extratruncal in 4) were as follows: 2 thyroid papillary cell carcinomas, 3 solitary lymph nodes with unknown primary (2 adenocarcinomas and 1 small cell carcinoma), 1 tonsil squamous cell carcinoma, 3 lung carcinomas (1 adenocarcinoma, 1 small cell, and 1 squamous cell), and 1 colon adenocarcinoma. Detection of a well-characterized neuronal nuclear or cytoplasmic paraneoplastic autoantibody was associated with a successful PET-CT–directed cancer search (P < .001). Detection of limited-stage cancer facilitated early initiation of oncologic treatments and immunotherapy; cancer remission was reported in 7 patients, and sustained improvements in neurologic symptoms were reported in 5 (median follow-up, 11 months; range, 2-48 months). Combined data from 2 previous studies using conventional PET alone (123 patients) revealed that 28% of patients had a PET abnormality suggestive of cancer and that 12% had a cancer diagnosis.
Conclusion In a paraneoplastic neurologic context, PET-CT improves the detection of cancers when other screening test results are negative, particularly in the setting of seropositivity for a neuronal nuclear or cytoplasmic autoantibody marker of cancer.
AUTHORS Andrew McKeon, MB, MRCPI; Metha Apiwattanakul, MD; Daniel H. Lachance, MD; Vanda A. Lennon, MD, PhD; Jayawant N. Mandrekar, PhD; Bradley F. Boeve, MD; Brian Mullan, MD; Bahram Mokri, MD; Jeffrey W. Britton, MD; Daniel A. Drubach, MD; Sean J. Pittock, MD all of Mayo Clinic.
Dr. Amy Krambeck discusses the advantages and outcomes for holmium laser enucleation of the prostate (HoLEP) performed for benign prostatic hyperplasia (BPH).
HoLEP was first performed in the United States in 1998; however, has been slow to gain widespread acceptance due to its steep learning curve. The procedure has been shown to have superior short and long-term outcomes to transurethral resection of the prostate (TURP) and suprapubic prostatectomy.
Benefits of the HoLEP procedure include complete removal of the adenoma to the level of the prostate resulting in a less than 2% retreatment rate, lack of surgical incision, and no effect on erectile function. Furthermore, HoLEP can be performed on prostate glands of any size. HoLEP is performed as an outpatient procedure or as a 23 hour observation and the urethral catheter is removed the day after surgery. All patients experience hematuria for 1 to 2 weeks post procedure, but the need for blood transfusion is 1%. Transient urinary incontinence is common, but permanent incontinence at 1 year post procedure occurs in approximately 1% of patients. We are now offering HoLEP at Mayo Clinic in Minnesota.
Safety, feasibility, and short-term outcomes of laparoscopically assisted primary ileocolic resection for Crohn’s disease
ABSTRACT Background Outcomes of laparoscopic resection for ileocecal Crohn’s disease have been reported previously in smaller studies, suggesting its short-term advantages over open surgery. This study assessed the safety and recovery parameters in the largest, consecutive, single-institution series to date.
Methods Consecutive patients undergoing laparoscopically assisted primary ileocolic resection for Crohn’s disease between 1994 and 2006 were identified in an institutional prospectively collected database. Operative and postoperative outcomes at 30 days were studied.
Results In this study, 109 patients (35 men) with a mean age of 35 ± 14 years and a mean body mass index (BMI) of 25 ± 6 kg/m2 were identified. The main indications for surgery were medically refractory disease (63%) and fibrous stenosis (27%). In 41% of the cases, previous abdominal surgery had been performed. The surgery had a mean duration of 150 ± 45 min and a conversion rate of 6%. The overall 30-day morbidity rate was 11%, and the reoperation rate was 1%. The mortality rate was 0%. The median postoperative hospital stay was 4 days (range, 2-15 days).
Conclusions This series, the largest reported to date, concurs with recent metaanalyses findings that laparoscopically assisted primary ileocecal resection for Crohn’s disease is safe and feasible, resulting in better short-terms outcomes than open resection. This operation is therefore the procedure of choice for Crohn’s disease at our institutions.
Authors
Mattias Soop, David W. Larson, Kishore Malireddy, Robert R. Cima, Tonia M. Young-Fadok, Eric J. Dozois all from Mayo Clinic
Dr. Bobbie Gostout provides a step by step commentary of a robotic hysterectomy. The procedure shown is a risk reducing hysterectomy and bilateral sub-oophorectomy for a woman with a BRCA mutation.
Dr. Elson So discusses a new technique known as STATISCOM to identify seizure origins in the brain. The increased accuracy of STATISCOM over the SISCOM technique, used previously, improves surgical accuracy and increases probability of seizure freedom for patients with epilepsy. The new technique will be described in the January 2010 issue of Neurology.
Abstract Objective: Although subtraction ictal SPECT coregistered to MRI (SISCOM) is clinically useful in epilepsy surgery evaluation, it does not determine whether the ictal-interictal subtraction difference is statistically different from the expected random variation between two SPECT studies. We developed a statistical parametric mapping and MRI voxel-based method of analyzing ictal-interictal SPECT difference data (statistical ictal SPECT coregistered to MRI [STATISCOM]) and compared it with SISCOM.
Methods: Two serial SPECT studies were performed in 11 healthy volunteers without epilepsy (control subjects) to measure random variation between serial studies from individuals. STATISCOM and SISCOM images from 87 consecutive patients who had ictal SPECT studies and subsequent temporal lobectomy were assessed by reviewers blinded to clinical data and outcome.
Results: Interobserver agreement between blinded reviewers was higher for STATISCOM images than for SISCOM images (=0.81 vs =0.36). STATISCOM identified a hyperperfusion focus in 84% of patients, SISCOM in 66% (P.05). STATISCOM correctly localized the temporal lobe epilepsy (TLE) subtypes (mesial vs lateral neocortical) in 68% of patients compared with 24% by SISCOM (P=.02); subgroup analysis of patients without lesions (as determined by MRI) showed superiority of STATISCOM (80% vs 47%; P=.04). Moreover, the probability of seizure-free outcome was higher when STATISCOM correctly localized the TLE subtype than when it was indeterminate (81% vs 53%, P=.03).
Conclusion: STATISCOM was superior to SISCOM for seizure localization before TLE surgery. STATISCOM localization to the correct TLE subtype was prognostically important for postsurgical seizure freedom.
Authors
N. J. Kazemi, MBBS, G. A. Worrell, MD, PhD, S. M. Stead, MD, PhD, B. H. Brinkmann, PhD, B. P. Mullan, MD, T. J. O’Brien, MD and E. L. So, MD From the Departments of Medicine, Surgery, and Neurology (N.J.K., T.J.O.), Royal Melbourne Hospital, University of Melbourne, Victoria, Australia; the Departments of Neurology (G.A.W., S.M.S., E.L.S.) and Radiology (B.P.M.), Mayo Clinic, Rochester, MN; and 3D Medical Imaging LLC (B.H.B.), Byron, MN
Dr. Ronald Peterson discusses a review paper in the Archives of Neurology summarizing both the progress that has been made in the field of MCI and the challenges that remain.
ABSTRACT In the past 10 years, there has been a virtual explosion in the literature concerning the construct of mild cognitive impairment. The interest in this topic demonstrates the increasing emphasis on the identification of the earliest features of cognitive disorders such as Alzheimer disease and other dementias. Mild cognitive impairment represents the earliest clinical features of these conditions and, hence, has become a focus of clinical, epidemiologic, neuroimaging, biomarker, neuropathological, disease mechanism, and clinical trials research. This review summarizes the progress that has been made while also recognizing the challenges that remain.
Authors
Ronald C. Petersen; Rosebud O. Roberts; David S. Knopman; Bradley F. Boeve; Yonas E. Geda; Robert J. Ivnik; Glenn E. Smith; Clifford R. Jack Jr from Mayo Clinic
Dr. Thomas Witzig, Dr. Matthew Drake, and Dr. James Cerhan discuss a new study that found the amount of vitamin D in patients being treated for diffuse large B-cell lymphoma was strongly associated with cancer progression and overall survival. The results were presented at the 2009 annual meeting of the American Society of Hematology .
ABSTRACT Background: Vitamin D is a naturally occurring steroid hormone. In addition to its well established role in maintaining serum calcium homeostasis, vitamin D has effects on cellular differentiation, proliferation, apoptosis, and angiogenesis. Vitamin D3 is naturally produced in skin in response ultraviolet-B (UVB) radiation from the sun. Several recent studies, however, have shown that a high proportion of community-dwelling subjects in both tropical and temperate climates are deficient in vitamin D, and that subjects in northern latitudes often require dietary supplementation to maintain vitamin D sufficiency. Further, several reports now suggest that vitamin D sufficiency is protective against the development of several cancers, including non-Hodgkin lymphoma (NHL). However, it is not known whether vitamin D impacts prognosis in diffuse large B-cell lymphoma (DLBCL), the most common NHL subtype.
Methods: We evaluated serum vitamin D concentrations in two cohorts of DLBCL patients. The first cohort consisted of 374 patients newly diagnosed from September 2002-February 2008 who were prospectively enrolled in the University of Iowa/Mayo Clinic Lymphoma SPORE Molecular Epidemiology Resource, an observational study in which neither therapeutic nor diagnostic management is prescribed. All serum was obtained within 120 days of diagnosis, and was prior to treatment in 221 (59%). In the second study, 62 patients newly diagnosed from February 2006-August 2007 were enrolled on NCCTG clinical trial N0489, and all serum was obtained before the first course of therapy. Central pathology review was performed on all patients to confirm the diagnosis of DLBCL. Serum 25-hydroxyvitamin (25-OH-VitD) levels were measured by the gold standard method for vitamin D determination: liquid chromatography tandem mass spectroscopy (LC-MS/MS). Vitamin D deficiency was defined as total serum 25-OH-VitD < 25 ng/mL (62.5 nmol/L). SPORE patients were followed per a standard protocol for event-free (progression, retreatment, or death due to any cause) and overall survival (EFS and OS, respectively); N0489 patients were followed in a similar way per the clinical trial protocol. We used Cox regression to estimate hazard ratios (HRs) and 95% confidence intervals (CI).
Results: SPORE patients were primarily treated with immunochemotherapy (83%); all N0489 patients were treated with epratuzumab + R-CHOP21. The median age at diagnosis was 62 years (range, 19-93) for SPORE patients and 61 years (range, 21-82) for N0489 patients. Median follow-up for SPORE patients was 36 months (range, 1-77) with 95 deaths and 131 events; median follow-up for N0489 patients was 24 months (range, 7-39) with 13 deaths and 18 events. Vitamin D deficiency was identified in 188 SPORE patients (50%) and 15 N0489 patients (24%). There were no differences in the prevalence of vitamin D deficiency by age, IPI, or whether serum was obtained prior to starting treatment (all p > 0.28). Vitamin D deficiency was associated with inferior overall (HR=2.33, 95% CI 1.53-3.57, logrank p =0.0001) and event-free survival (HR=1.71, 95% CI 1.20-2.44, logrank p = 0.003) in the SPORE cohort. These associations remained significant after adjusting for IPI and treatment: OS HR=1.97, 95% CI 1.27-3.07; EFS HR=1.47, 95% CI 1.02-2.21. These results were similar for the subset of SPORE patients who were treated with R-CHOP. The HRs for vitamin D deficiency and event-free survival were consistent in the N0489 patients (HR=1.63, 95% CI 0.61-4.35, IPI adjusted HR=1.43, 95% CI 0.53-3.85), although these results lacked precision due to a smaller sample size. We were unable to evaluate OS in N0489 due to the small number of deaths.
Conclusions: Approximately 50% of all DLBCL patients in this northern US latitude population are vitamin D deficient at the time of diagnosis and treatment. Vitamin D deficient patients have an inferior event-free and overall survival compared to patients with vitamin D levels within the normal range. Vitamin D supplementation during treatment of DLBCL patients with vitamin D deficiency should be evaluated in a clinical trial setting.
Authors
Matthew T Drake, MD, PhD1*, Matthew J Maurer, MS1*, Brian K Link, MD2*, Ivana N Micallef, MD1*, Thomas M Habermann, MD1, Jennifer L Kelly, PhD, MPH3, William R Macon, MD1*,
Daniel Nikcevich, MD4*, Joseph P Colgan, MD1*, Cristine Allmer, BS1*, Susan L Slager, PhD1*, George J Weiner, MD2, Thomas E Witzig, MD1* and James R Cerhan, MD, PhD1
1Mayo Clinic, Rochester, MN; 2University of Iowa, Iowa City, IA;
3University of Rochester, Rochester, NY; 4St. Mary’s Duluth Clinic, Duluth, MN
Mayo Clinic research regarding children and anesthesia:
Arendt KW, Haile DT, Rose CH, Craigo PA, Flick RP, Chantigian RC. Prolonged exit procedure and the analysis of umbilical cord gases. Anesthesiology 2007 May; 106(5):B98. (Abstract)
*Haile DT, Orvidas LJ, Weaver A, Li X, Flick RP. The association of age and increased body mass index among Olmsted County children undergoing (adeno)-tonsillectomy for sleep disordered breathing. American Society of Anesthesiologists Annual Meeting, San Francisco, CA October 2007. (Abstract)
Wilder RT, Flick RP, Sprung J, Warner DO, Katusic SK. Relationship between exposure to anesthesia and subsequent learning disabilities in children. Anesthesiology 2008; 109:A1416. (Abstract)
Haile DT, Arendt KW, Flick RP. The intraoperative management of a patient with fontan physiology and an undiagnosed pheochromocytoma. Paediatr Anaesth 2008 Apr; 18(4):353-5. PMID:18315660. DOI:10.1111/j.1460-9592.2008.02443.x. (Letter)
*Kenyon CA, Flick RP, Moir C, Ackerman MJ, Pabelick CM. Anesthesia for cardiac sympathectomy in children with congenital long QT syndrome: A case series. Society for Pediatric Anesthesia Spring Meeting 2009. (Abstract)
*Rabbitts JA, Flick RP. Ambulatory anesthesia in the pediatric population of the USA. Society of Pediatric Anesthesia Jacksonville, FL. March 2009. (Abstract)