Intestinal malrotation in the adult population: Diagnosis, management, and outcomes after laparoscopic Ladd procedure.

BACKGROUND: The Ladd procedure is the treatment of choice for patients with intestinal malrotation; however, the long-term outcomes of the laparoscopic Ladd procedure are not well documented. This study aimed to review the presentation, management, and outcomes of adult patients who underwent a laparoscopic Ladd procedure. METHODS: A retrospective review was conducted to identify adult patients with malrotation who underwent a laparoscopic Ladd procedure between January 1995 and June 2022 at the Mayo Clinic in Rochester, Minnesota. Patient details and follow-up data were obtained from the electronic medical records. Patients were invited to participate in a structured phone interview to assess symptoms and quality of life (QoL). RESULTS: A total of 44 patients underwent the laparoscopic Ladd procedure. Of the 44 patients, 42 (95.5 %) were symptomatic, with 7 (16.7 %) presenting with acute symptoms. Moreover, 8 laparoscopic procedures (13.6 %) required conversion to an open procedure. The median estimated blood loss was 20 mL (IQR, 10-50), operative time was 2.3 h (IQR, 1.8-2.8), and hospital length of stay was 2 days (IQR, 2-3). Postoperative ileus was the most common complication (18.0 %). The median follow-up was 8.00 years (IQR, 2.25-13.00), with more than 90.0 % of patients having partial or complete symptom resolution. Of note, 28 patients (63.6 %) completed phone interviews. Moreover, 1 patient (2.0 %) reported a postoperative volvulus. When asked to compare their current symptoms with those preoperatively, 78.6 % of patients noted that they were significantly better. Furthermore, 85.7 % of patients reported that their QoL was significantly better after surgery. Finally, 96.4 % of patients would recommend the procedure to a friend or family member with the same condition. CONCLUSION: The laparoscopic Ladd procedure is a safe and effective surgical procedure for adult patients with intestinal malrotation.

Evolution of laparoscopic ileal pouch-anal anastomosis: impact of enhanced recovery program, medication changes, and staged approaches on outcomes.

BACKGROUND: Although laparoscopic Ileal pouch-anal anastomosis (IPAA) has become the gold standard in restorative proctocolectomy, surgical techniques have experienced minimal changes. In contrast, substantial shifts in perioperative care, marked by the enhanced recovery program (ERP), modifications in steroid use, and a shift to a 3-staged approach, have taken center stage. METHODS: Data extracted from our prospective IPAA database focused on the first 100 laparoscopic IPAA cases (historic group) and the latest 100 cases (modern group), aiming to measure the effect of these evolutions on postoperative outcomes. RESULTS: The historic IPAA group had more 2-staged procedures (92% proctocolectomy), whereas the modern group had a higher number of 3-staged procedures (86% proctectomy) (P < .001). Compared with patients in the modern group, patients in the historic group were more likely to be on steroids (5% vs 67%, respectively; P < .001) or immunomodulators (0% vs 31%, respectively; P < .001) at surgery. Compared with the historic group, the modern group had a shorter operative time (335.5 ± 78.4 vs 233.8 ± 81.6, respectively; P < .001) and length of stay (LOS; 5.4 ± 3.1 vs 4.2 ± 1.6 days, respectively; P < .001). Compared with the modern group, the historic group exhibited a higher 30-day morbidity rate (20% vs 33%, respectively; P = .04) and an elevated 30-day readmission rate (9% vs 21%, respectively; P = .02). Preoperative steroids use increased complications (odds ratio [OR], 3.4; P = .01), whereas 3-staged IPAA reduced complications (OR, 0.3; P = .03). ERP was identified as a factor that predicted shorter stays. CONCLUSION: Although ERP effectively reduced the LOS in IPAA surgery, it failed to reduce complications. Conversely, adopting a 3-staged IPAA approach proved beneficial in reducing morbidity, whereas preoperative steroid use increased complications.

Ischiorectal Fossa Tumors: Thirty-Year Single Institution Experience.

BACKGROUND: Primary tumors of the ischiorectal fossa are rare and comprise a wide array of pathologies with varying malignant potential. Due to the low prevalence, there is a paucity of data in the literature. This paper presents a case-series on the management of ischiorectal fossa tumors. OBJECTIVE: To present a 30-year experience managing ischiorectal fossa tumors. DESIGN: Retrospective single center analysis. SETTINGS: A quaternary referral academic healthcare center. PATIENTS: All patients treated for ischiorectal fossa tumors. INTERVENTIONS: All patients underwent surgical management of their disease. MAIN OUTCOME MEASURES: Disease recurrence and overall survival. RESULTS: A total of 34 patients (53% female) were identified with a median follow-up of 23 months. Twenty-one patients (62%) were diagnosed with benign and 13 (38%) with malignant tumors. All underwent surgical resection. Median tumor size was 8.4 cm. R0 resection was obtained in 28 patients. Twelve (35%) developed recurrence (nine following R0 resection) with a median time of 6.5 months. There were no surgical related mortalities. LIMITATIONS: Limitations to the study include its retrospective nature, single center experience, and small patient sample size. CONCLUSIONS: Ischiorectal fossa tumors are primarily benign, however they are associated with high recurrence rates even in the setting of an R0 resection. Treatment should be approached in a multidisciplinary fashion and preferably in centers with experience treating these tumors. Close post treatment surveillance is imperative. See Video Abstract.

Presacral Neuroendocrine Neoplasms: A Multi-site Review of Surgical Outcomes.

INTRODUCTION: Presacral neuroendocrine neoplasms (PNENs) are rare tumors, with limited data on management and outcomes. METHODS: A retrospective review of institutional medical records was conducted to identify all patients with PNENs between 2008 and 2022. Data collection included demographics, symptoms, imaging, surgical approaches, pathology, complications, and long-term outcomes. RESULTS: Twelve patients were identified; two-thirds were female, averaging 44.8 years of age, and, for the most part, presenting with back pain, constipation, and abdominal discomfort. Preoperative imaging included computed tomography scans and magnetic resonance images, with somatostatin receptor imaging and biopsies being common. Half of the patients had metastatic disease on presentation. Surgical approach varied, with anterior, posterior, and combined techniques used, often involving muscle transection and coccygectomy. Short-term complications affected one-quarter of patients. Pathologically, PNENs were mainly well-differentiated grade 2 tumors with positive synaptophysin and chromogranin A. Associated anomalies were common, with tail-gut cysts prevalent. Mean tumor diameter was 6.3 cm. Four patients received long-term adjuvant therapy. Disease progression necessitated additional interventions, including surgery and various chemotherapy regimens. Skeletal, liver, thyroid, lung, and pancreatic metastases occurred during follow-up, with no mortality reported. Kaplan-Meier analysis showed a 5-year local recurrence rate of 23.8%, disease progression rate of 14.3%, and de novo metastases rate of 30%. CONCLUSION: The study underscores the complex management of PNENs and emphasizes the need for multicenter research to better understand and manage these tumors. It provides valuable insights into surgical outcomes, recurrence rates, and overall survival, guiding future treatment strategies for PNEN patients.

The Impact of Enhanced Recovery on Long-Term Survival in Rectal Cancer.

INTRODUCTION: Implementing perioperative interventions such as enhanced recovery pathways (ERPs) has improved short-term outcomes and minimized length of stay. Preliminary evidence suggests that adherence to the enhanced recovery after surgery protocol may also enhance 5-year cancer-specific survival (CSS) in colorectal cancer surgery. This retrospective study presents long-term survival outcomes and disease recurrence from a high-volume, single-center practice. METHODS: All patients over 18 years of age diagnosed with rectal adenocarcinoma and undergoing elective minimally invasive surgery (MIS) were retrospectively reviewed between February 2005 and April 2018. Relevant data were extracted from Mayo electronic records and securely stored in a database. Short-term morbidity and long-term oncological outcomes were compared between patients enrolled in ERP and those who received non-enhanced care. RESULTS: Overall, 600 rectal cancer patients underwent MIS, of whom 320 (53.3%) were treated according to the ERP and 280 (46.7%) received non-enhanced care. ERP was associated with a decrease in length of stay (3 vs. 5 days; p < 0.001) and less overall complications (34.7 vs. 54.3%; p < 0.001). The ERP group did not show an improvement in overall survival (OS) or disease-free survival (DFS) compared with non-enhanced care on multivariable (non-ERP vs. ERP OS: hazard ratio [HR] 1.268, 95% confidence interval [CI] 0.852-1.887; DFS: HR 1.050, 95% CI 0.674-1.635) analysis. CONCLUSION: ERP was found to be associated with a reduction in short-term morbidity, with no impact on long-term oncological outcomes, such as OS, CSS, and DFS.

Aggressive Angiomyxoma of the Pelvis: 35-Year Experience.

BACKGROUND: Aggressive angiomyxoma is a very rare mesenchymal tumor most commonly found in the pelvic and perineal regions. Although many are estrogen and progesterone hormone receptor positive, the pathogenesis is unknown. Due to its rarity, there is a paucity of literature relating to this pathology. This article presents a case series on the management of aggressive angiomyxoma of the pelvis. OBJECTIVE: To present a 35-year experience managing aggressive angiomyxoma of the pelvis. DESIGN: This was a retrospective single-system analysis. SETTINGS: This study was conducted at a quaternary referral academic health care system. PATIENTS: All patients treated for aggressive angiomyxoma of the pelvis. INTERVENTIONS: All patients underwent surgical or medical management of their disease. MAIN OUTCOME MEASURES: The primary outcomes were disease recurrence and mortality. Secondary outcomes included risk factors for recurrence. RESULTS: A total of 32 patients (94% women) were identified with a median follow-up of 65 months. Thirty patients (94%) underwent operative resection and 2 patients were treated solely with medical management. Fifteen achieved an R0 resection (negative microscopic margins) at the index operation, of which 4 (27%) experienced tumor recurrence. There were no mortalities. No risk factors for disease recurrence were identified. LIMITATIONS: Limitations to our study include its nonrandomized retrospective nature, single health care system experience, and small patient sample size. CONCLUSIONS: Aggressive angiomyxoma is a rare, slow-growing tumor with locally invasive features and a high potential for recurrence even after resection with negative margins. Imaging modalities such as CT or MRI should be obtained to aid in diagnosis and surgical planning. Workup should be paired with preoperative biopsy and testing for hormone receptor status, which can increase diagnostic accuracy and guide medical treatment. Close posttreatment surveillance is imperative to detect recurrence. See Video Abstract . ANGIOMIXOMA AGRESIVO DE PELVIS EXPERIENCIA DE AOS: ANTECEDENTES:El angiomixoma agresivo es un tumor mesenquimal muy raro que se encuentra más comúnmente en las regiones pélvica y perineal. Aunque muchos son positivos para los receptores hormonales como el estrógeno y la progesterona, la patogénesis es aún desconocida. Debido a su rareza, existe escasa literatura relacionada con esta patología. Este artículo presenta una serie de casos sobre el tratamiento del angiomixoma agresivo de pelvis.OBJETIVO:Presentar una experiencia de 35 años en el manejo del angiomixoma agresivo de pelvis.DISEÑO:Este fue un análisis retrospectivo de sistema único.AJUSTES:Este estudio se llevó a cabo en un sistema de salud académico de referencia de nivel cuaternario.PACIENTES:Todos los pacientes tratados por angiomixoma agresivo de pelvis.INTERVENCIONES:Todos los pacientes se sometieron a tratamiento quirúrgico y/o médico de su enfermedad.PRINCIPALES MEDIDAS DE RESULTADO:Los resultados primarios fueron la recurrencia de la enfermedad y la mortalidad. Los resultados secundarios incluyeron factores de riesgo de recurrencia.RESULTADOS:Se identificaron un total de 32 pacientes (94% mujeres) con una mediana de seguimiento de 65 meses. Treinta (94%) fueron sometidos a resección quirúrgica y dos fueron tratados únicamente con tratamiento médico. Quince lograron una resección R0 (márgenes microscópicos negativos) en la operación inicial, de los cuales cuatro (27%) experimentaron recurrencia tumoral. No hubo mortalidades. No se identificaron factores de riesgo para la recurrencia de la enfermedad.LIMITACIONES:Las limitaciones de nuestro estudio incluyen su naturaleza retrospectiva no aleatoria, la experiencia de un solo sistema de atención médica y el tamaño pequeño de la muestra de pacientes.CONCLUSIONES:El angiomixoma agresivo es un tumor raro, de crecimiento lento, con características localmente invasivas y un alto potencial de recurrencia incluso después de una resección con márgenes negativos. Se deben obtener modalidades de imágenes como CT y/o MRI para la ayuda diagnóstica y la planificación quirúrgica. El estudio debe combinarse con una biopsia preoperatoria y pruebas del estado de los receptores hormonales, que pueden aumentar la precisión del diagnóstico y guiar el tratamiento médico. Es imperativa una estrecha vigilancia posterior al tratamiento para detectar recurrencia. (Traducción-Dr Osvaldo Gauto ).

MR Defecating Proctography with Emphasis on Posterior Compartment Disorders.

MR defecating proctography (MRDP) is a noninvasive examination that can be used for evaluating posterior compartment disorders. MRDP has several advantages over conventional fluoroscopic defecography. These benefits include high-contrast resolution evaluation of the deep pelvic organs, simultaneous multicompartmental assessment that is performed statically and dynamically during defecation, and lack of ionizing radiation. MRDP also provides a highly detailed anatomic evaluation of the pelvic floor supportive structures, including direct assessment of the pelvic floor musculature and indirect assessment of the endopelvic fascia. As the breadth of knowledge regarding anatomic and functional posterior compartment disorders expands, so too does the advancement of noninvasive and surgical treatment options for these conditions. High-quality MRDP examinations, with key anatomic and functional features reported, guide treatment planning. Reporting of MRDP examination findings with use of standardized terminology that emphasizes objective measurements rather than subjective grading aids consistent communication among radiologists, clinicians, and surgeons. Familiarity with commonly encountered posterior compartment pelvic floor pathologic entities that contribute to posterior compartment disorders and awareness of the essential information needed by surgeons are key to providing an optimal multidisciplinary discussion for planning pelvic floor dysfunction treatment. The authors provide an overview of the basic concepts of the MRDP acquisition technique, the anatomic abnormalities of posterior compartment pelvic floor pathologic entities associated with defecatory disorders, and recently developed interdisciplinary MRDP reporting templates and lexicons. In addition, the associated imaging findings that are key for surgical treatment guidance are highlighted. © RSNA, 2022 Online supplemental material is available for this article.

Re-resection of Microscopically Positive Margins Found on Intraoperative Frozen Section Analysis Does Not Result in a Survival Benefit in Patients Undergoing Surgery and Intraoperative Radiation Therapy for Locally Recurrent Rectal Cancer.

BACKGROUND: Intraoperative frozen-section analysis provides real-time margin resection status that can guide intraoperative decisions made by the surgeon and radiation oncologist. For patients with locally recurrent rectal cancer undergoing surgery and intraoperative radiation therapy, intraoperative re-resection of positive margins to achieve negative margins is common practice. OBJECTIVE: This study aimed to assess whether re-resection of positive margins found on intraoperative frozen-section analysis improves oncologic outcomes. DESIGN: This is a retrospective cohort study. SETTINGS: This study was an analysis of a prospectively maintained multicenter database. PATIENTS: All patients who underwent surgical resection of locally recurrent rectal cancer with intraoperative radiation therapy between 2000 and 2015 were included and followed for 5 years. Three groups were compared: initial R0 resection, initial R1 converted to R0 after re-resection, and initial R1 that remained R1 after re-resection. Grossly positive margin resections (R2) were excluded. MAIN OUTCOME MEASURES: The primary outcome measures were 5-year overall survival, recurrence-free survival, and local re-recurrence. RESULTS: A total of 267 patients were analyzed (initial R0 resection, n = 94; initial R1 converted to R0 after re-resection, n = 95; initial R1 that remained R1 after re-resection, n = 78). Overall survival was 4.4 years for initial R0 resection, 2.7 years for initial R1 converted to R0 after re-resection, and 2.9 years for initial R1 that remained R1 after re-resection ( p = 0.01). Recurrence-free survival was 3.0 years for initial R0 resection and 1.8 years for both initial R1 converted to R0 after re-resection and initial R1 that remained R1 after re-resection ( p ≤ 0.01). Overall survival did not differ for patients with R1 and re-resection R1 or R0 ( p = 0.62). Recurrence-free survival and freedom from local re-recurrence did not differ between groups. LIMITATIONS: This study was limited by the heterogeneous patient population restricted to those receiving intraoperative radiation therapy. CONCLUSIONS: Re-resection of microscopically positive margins to obtain R0 status does not appear to provide a significant survival advantage or prevent local re-recurrence in patients undergoing surgery and intraoperative radiation therapy for locally recurrent rectal cancer. See Video Abstract at http://links.lww.com/DCR/B886 . LA RERESECCIN DE LOS MRGENES MICROSCPICAMENTE POSITIVOS ENCONTRADOS DE MANERA INTRAOPERATORIA MEDIANTE LA TCNICA DE CRIOSECCIN, NO DA COMO RESULTADO UN BENEFICIO DE SUPERVIVENCIA EN PACIENTES SOMETIDOS A CIRUGA Y RADIOTERAPIA INTRAOPERATORIA PARA EL CNCER RECTAL LOCALMENTE RECIDIVANTE: ANTECEDENTES:El análisis de la ténica de criosección para los margenes positivos encontrados de manera intraoperatoria proporciona el estado de la resección del margen en tiempo real que puede guiar las decisiones intraoperatorias tomadas por el cirujano y el oncólogo radioterapeuta. Para los pacientes con cáncer de recto localmente recurrente que se someten a cirugía y radioterapia intraoperatoria, la re-resección intraoperatoria de los márgenes positivos para lograr márgenes negativos es una práctica común.OBJETIVO:Evaluar si la re-resección de los márgenes positivos encontrados en el análisis de la ténica por criosecciónde manera intraoperatorios mejora los resultados oncológicos.DISEÑO:Estudio de cohorte retrospectivo.AJUSTES:Análisis de una base de datos multicéntrica mantenida de forma prospectiva.POBLACIÓN:Todos los pacientes que se sometieron a resección quirúrgica de cáncer de recto localmente recurrente con radioterapia intraoperatoria entre 2000 y 2015 fueron incluidos y seguidos durante 5 años. Se compararon tres grupos: resección inicial R0, R1 inicial convertido en R0 después de la re-resección y R1 inicial que permaneció como R1 después de la re-resección. Se excluyeron las resecciones de márgenes macroscópicamente positivos (R2).PRINCIPALES MEDIDAS DE RESULTADO:Supervivencia global a cinco años, supervivencia sin recidiva y recidiva local.RESULTADOS:Se analizaron un total de 267 pacientes (resección inicial R0 n = 94, R1 inicial convertido en R0 después de la re-resección n = 95, R1 inicial que permaneció como R1 después de la re-resección n = 78). La supervivencia global fue de 4,4 años para la resección inicial R0, 2,7 años para la R1 inicial convertida en R0 después de la re-resección y 2,9 años para la R1 inicial que permaneció como R1 después de la re-resección ( p = 0,01). La supervivencia libre de recurrencia fue de 3,0 años para la resección inicial R0 y de 1,8 años para el R1 inicial convertido en R0 después de la re-resección y el R1 inicial que permaneció como R1 después de la re-resección ( p ≤ 0,01). La supervivencia global no difirió para los pacientes con R1 y re-resección R1 o R0 ( p = 0,62). La supervivencia libre de recurrencia y la ausencia de recurrencia local no difirieron entre los grupos.LIMITACIONES:Población de pacientes heterogénea, restringida a aquellos que reciben radioterapia intraoperatoria.CONCLUSIONES:La re-resección de los márgenes microscópicamente positivos para obtener el estado R0 no parece proporcionar una ventaja de supervivencia significativa o prevenir la recurrencia local en pacientes sometidos a cirugía y radioterapia intraoperatoria para el cáncer de recto localmente recurrente. Consulte Video Resumen en http://links.lww.com/DCR/B886 . (Traducción-Dr. Daniel Guerra ).

Sexual dysfunction following surgery for rectal cancer: a single-institution experience.

Although much focus is placed on oncological outcomes for rectal cancer, it is important to assess quality of life after surgery of which sexual function is an important component. This study set about to describe the prevalence of sexual dysfunction by resection type and gender among patients undergoing surgery for rectal cancer, usingretrospective analysis. All English-speaking living patients who underwent surgery for stage I-III rectal cancer with curative intent between 2012 and 2016 were identified from a prospectively maintained database at our institution. Eligible patients were invited to complete either the Female Sexual Function Index (FSFI) or the International Index of Erectile Function (IIEF). Primary outcomes were overall rates of sexual dysfunction, defined as more than one standard deviation below the mean of the normal population for each tool. A total of 147 patients responded, yielding a response rate of 38%. The overall sexual dysfunction rate was 70% at a median time from surgery of 38 months. Sixty-two men (62%) and 41 women (87%) reported overall scores that fell below one standard deviation of the population mean. There was no significant difference in sexual dysfunction for both male and female patients between low anterior resection, coloanal anastomosis, or abdominoperineal resection.. The present study revealed a high rate of sexual dysfunction after rectal cancer surgery, particularly in female patients. This study serves as a reminder to surgeons and their teams to openly discuss the impact of surgery on sexual function and ensure adequate consent and appropriate peri-operative management strategies. The retrospective nature of the analysis is the limitation of this study.

The extent of colorectal resection and short-term outcomes in patients with ulcerative colitis.

OBJECTIVE: There is limited literature on the impact of the extent of resection on short-term outcomes in patients with ulcerative colitis (UC) in an elective setting. The aim of this study was to better understand the impact of approach and extent of resection on short-term outcomes for patients undergoing total proctocolectomy (TPC) and subtotal colectomy (STC) for UC. METHODS: Patients with UC who underwent elective TPC or STC were captured from the ACS-NSQIP® 2011-2018 database and divided into four cohorts: Open TPC (O-TPC), Laparoscopic TPC (L-STC), Open STC (O-STC), and Laparoscopic STC (L-STC). Baseline and perioperative variables were compared between the four groups alongside 30-day mortality and 30-day complication rates. RESULTS: Of 3387 patients, 368 (10.9%) underwent O-STC, 406 (12%) underwent O-TPC, 1958 (58%) underwent L-STC, and 655 (19%) underwent L-TPC. Overall rate of prolonged length of stay (LOS) was 27% and 9% needed a blood transfusion. There was no difference in the risk of complications between open TPC and open STC. Those who had open surgery had a higher risk of complications and prolonged LOS. Patients who had L-TPC had prolonged LOS compared to patients who had L-STC, but less compared to those who had O-STC. CONCLUSION: Elective surgery for UC is associated with high rates of prolonged LOS and blood transfusion despite MIS approaches. Short-term outcomes and LOS are more impacted by the operative approach than the extent of resection. Despite this laparoscopic TPC has higher rates of prolonged LOS when compared to laparoscopic STC.

Predicting primary postoperative pulmonary complications in patients undergoing minimally invasive surgery for colorectal cancer.

OBJECTIVE: To determine the risk factors for developing primary postoperative pulmonary complications (PPC) in patients undergoing minimally invasive colorectal surgery (MIS) for the treatment of cancer and to identify the potential indicators for more extensive preoperative evaluation. MATERIALS AND METHODS: The ACS-NSQIP® database was interrogated to capture patients who had elective colon or rectal cancer and underwent MIS between 2012 and 2017. Patients who had primary PPC including pneumonia, unplanned intubation and/or failure to wean from mechanical ventilation for > 48 h were compared to patients without PPC. Significant risk factors for PPC were retained to build a predictive risk model through logistic regression analysis. The model was then internally validated using 2018 data. RESULTS: Of 50,150 patients identified, 637 (1.3%) had PPC. The final risk prediction model included six variables: history of chronic obstructive pulmonary disease, age, smoking status, functional health status, pre-operative congestive heart failure, and American Society of Anesthesiology class ≥ 3. The model achieved good calibration (Hosmer-Lemeshow goodness-of-fit test, p = 0.614) and discrimination (c statistics = 0.757). Internal validation achieved similar discrimination (c statistics = 0.798). CONCLUSION: Primary postoperative pulmonary complications affected 1.3% of patients undergoing MIS for colon or rectal cancer. The novel predictive risk score showed good discrimination and may help to identify patients who may benefit from perioperative optimization.

Multiple system atrophy pathology is associated with primary Sjögren's syndrome.

BACKGROUNDOur objective was to investigate whether primary Sjögren's syndrome (pSS) is associated with multiple system atrophy (MSA).METHODSWe performed a retrospective cohort study assessing (a) rates of MSA in a cohort of patients with pSS and (b) rates of pSS in a cohort of patients with MSA. These data were compared with rates in respective control groups. We additionally reviewed the neuropathologic findings in 2 patients with pSS, cerebellar degeneration, parkinsonism, and autonomic dysfunction.RESULTSOur cohort of 308 patients with pSS had a greater incidence of MSA compared with 4 large population-based studies and had a significantly higher prevalence of at least probable MSA (1% vs. 0%, P = 0.02) compared with 776 patients in a control cohort of patients with other autoimmune disorders. Our cohort of 26 autopsy-proven patients with MSA had a significantly higher prevalence of pSS compared with a cohort of 115 patients with other autopsy-proven neurodegenerative disorders (8% vs. 0%, P = 0.03). The 2 patients we described with pSS and progressive neurodegenerative disease showed classic MSA pathology at autopsy.CONCLUSIONOur findings provide evidence for an association between MSA and pSS that is specific to both pSS, among autoimmune disorders, and MSA, among neurodegenerative disorders. The 2 cases we describe of autopsy-proven MSA support that MSA pathology can explain neurologic disease in a subset of patients with pSS. These findings together support the hypothesis that systemic autoimmune disease plays a role in neurodegeneration.FUNDINGThe Michigan Brain Bank is supported in part through NIH grant P30AG053760.

Delivery of surgical care in Samoa: perspectives on capacity, barriers and opportunities by local providers.

BACKGROUND: The Pacific Island nation of Samoa faces a number of challenges in delivering surgical care. Our group aimed to identify the barriers and opportunities to improving the delivery of safe, affordable, timely surgical care in Samoa. METHODS: A mixed-methods approach was undertaken. The quantitative analysis used a modified version of the World Health Organization Emergency and Essential Surgical Checklist while the qualitative methodology used semi-structured interviews. Respondents were asked to share their views on the capacity, quality, accessibility and future directions of surgery in Samoa. Interviews were transcribed and analysed using open and axial coding techniques. RESULTS: Stakeholders had a positive outlook on the delivery of surgical care, but it was suggested that existing services were not meeting needs. Respondents cited limited access to equipment and resources, compounded by insufficient organizational and logistical infrastructure. Shortage of medical staff and retention was identified as a key issue. Shortcomings in primary care and poor health literacy were seen as significant barriers to accessing care. CONCLUSION: Documenting locally identified barriers and solutions to surgical care in Samoa is an important first step towards the development of formal strategies for improving surgical services nationally.

Collecting data for global surgical indicators: a collaborative approach in the Pacific Region.

In 2015, the Lancet Commission on Global Surgery (LCoGS) recommended six surgical metrics to enable countries to measure their surgical and anaesthesia care delivery. These indicators have subsequently been accepted by the World Bank for inclusion in the World Development Indicators. With support from the Royal Australasian College of Surgeons and the Pacific Islands Surgical Association, 14 South Pacific countries collaborated to collect the first four of six LCoGS indicators. Thirteen countries collected all four indicators over a 6-month period from October 2015 to April 2016. Australia and New Zealand exceeded the recommended LCoGS target for all four indicators. Only 5 of 13 countries (38%) achieved 2-hour access for at least 80% of their population, with a range of 20% (Papua New Guinea and Solomon Islands) to over 65% (Fiji and Samoa). Five of 13 (38%) countries met the target surgical volume of 5000 procedures per 100 000 population, with six performing less than 1600. Four of 14 (29%) countries had at least 20 surgical, anaesthesia and obstetric providers in their workforce per 100 000 population, with a range of 0.9 (Timor Leste) to 18.5 (Tuvalu). Perioperative mortality rate was reported by 13 of 14 countries, and ranged from 0.11% to 1.0%. We believe it is feasible to collect global surgery indicators across the South Pacific, a diverse geographical region encompassing high-income and low-income countries. Such metrics will allow direct comparison between similar nations, but more importantly provide baseline data that providers and politicians can use in advocacy national health planning.

Integrating Cerebrospinal Fluid Shunt Quality Checks into the World Health Organization's Safe Surgery Checklist: A Pilot Study.

BACKGROUND: Despite efforts for improvement, cerebrospinal fluid (CSF) shunt failure rates remain high. Recent studies have shown promising reductions in failure rates and infection rates with the routine use of perioperative checklists. This study was conducted to pilot test the feasibility and efficacy of integrating specific CSF shunt surgery quality checks into the World Health Organization (WHO) Surgical Safety Checklist. METHODS: We designed CSF shunt checklist quality items according to a previously established methodology, including solicitation of best practices by a national multidisciplinary expert panel. We examined adherence to key processes before and after implementation as a measure of the efficacy of the integrated checklist. We then surveyed users regarding perceived checklist utility. RESULTS: Overall adherence to shunt-specific key processes increased from 8.6 (95% confidence interval [CI], 7.9-9.2) to 9.9 (95% CI, 9.3-10.4; P = 0.0070) per 12 items, driven by the infection control items (4.7 [95% CI, 4.1-5.3] to 6.0 [95% CI, 5.4-6.4] per 8 items; P = 0.0056). All of the survey respondents indicated that the checklist was easy to use. The majority stated that it helped them feel better prepared to perform the procedure consistently according to evidence-based practice, and that if they were to adhere to the checklist consistently, their rate of shunt failure would be expected to decrease. CONCLUSIONS: The integration of specialty-specific checks into the WHO Safe Surgery Checklist improved adherence to quality processes and generally was well accepted in our pilot study. A larger clinical trial is needed to assess whether this approach could improve shunt outcomes.

Vitamin D from different sources is inversely associated with Parkinson disease.

An inverse association between Parkinson disease (PD) and total vitamin D levels has been reported, but whether vitamin D from different sources, that is, 25(OH)D2 (from diet and supplements) and 25(OH)D3 (mainly from sunlight exposure), all contribute to the association is unknown. Plasma total 25(OH)D, 25(OH)D2, and 25(OH)D3 levels were measured by liquid chromatography-tandem mass spectrometry in PD patients (n = 478) and controls (n = 431). Total 25(OH)D was categorized by clinical insufficiency or deficiency; 25(OH)D2 and 25(OH)D3 were analyzed in quartiles. Vitamin D deficiency (total 25[OH]D < 20 ng/mL) and vitamin D insufficiency (total 25[OH]D < 30 ng/mL) are associated with PD risk (odds ratio [OR] = 2.6 [deficiency] and 2.1 [insufficiency]; P < 0.0001), adjusting for age, sex, and sampling season. Both 25(OH)D2 and 25(OH)D3 levels are inversely associated with PD (P(trend) < 0.0001). The association between 25(OH)D2 and PD risk is largely confined to individuals with low 25(OH)D3 levels (P(trend) = 0.0008 and 0.12 in individuals with 25[OH]D3 < 20 ng/mL and 25[OH]D3 ≥ 20 ng/mL, respectively). Our data confirm the association between vitamin D deficiency and PD, and for the first time demonstrate an inverse association of 25(OH)D2 with PD. Given that 25(OH)D2 concentration is independent of sunlight exposure, this new finding suggests that the inverse association between vitamin D levels and PD is not simply attributable to lack of sunlight exposure in PD patients with impaired mobility. The current study, however, cannot exclude the possibility that gastrointestinal dysfunction, a non-motor PD symptom, contributes to the lower vitamin D2 levels in PD patients.