Crohn's-related enterocutaneous fistula: a unique cause of penile prosthesis infection.

Genitourinary manifestations are rare in patients with Crohn's disease, and a small percentage of patients will experience enterocutaneous fistulas. Infection is one of the most common complications associated with inflatable penile prosthesis placement, which can be associated with fistula formation. In this report, we present a patient with Crohn's disease who developed an inflatable penile prosthesis infection secondary to an undiagnosed enterocutaneous fistula.

Subclavian Steal Phenomenon Associated With Vascular Ring in an Infant Who Had a Prenatally Diagnosed Right Aortic Arch With an Atretic Aberrant Left Subclavian Artery.

Vascular ring is a rare congenital anomaly in which the abnormal origin of the aorta or its branches and pulmonary arteries leads to encircling and compression of the trachea and esophagus. A right aortic arch (RAA) with an aberrant left subclavian artery is one of the most common forms of vascular ring. Here, we report a case of a prenatally diagnosed vascular ring resulting from an RAA with an aberrant left subclavian artery. When the infant was 7 months of age, the development of noisy breathing prompted further evaluation with cardiac magnetic resonance imaging that showed an atretic left subclavian artery associated with collateral retrograde flow from the left vertebral artery to the distal portion of the subclavian artery. Our findings indicate that an untreated RAA with an aberrant left subclavian artery may be associated with an increased risk of developing subclavian artery steal syndrome.

Consensus statement on networks for high-quality rural anesthesia, surgery, and obstetric care in Canada.

OBJECTIVE: To describe the essential components of well-resourced and high-functioning multidisciplinary networks that support high-quality anesthesia, surgery, and maternity care for rural Canadians, delivered as close to home as possible. COMPOSITION OF THE COMMITTEE: A volunteer Writers' Group was drawn from the Society of Obstetricians and Gynaecologists of Canada, the Society of Rural Physicians of Canada, the Royal College of Physicians and Surgeons of Canada, the Canadian Association of General Surgeons, the College of Family Physicians of Canada, and the Association of Canadian University Departments of Anesthesia. METHODS: A collaborative effort over the past several years among the professional stakeholders has culminated in this consensus statement on networked care designed to integrate and support a specialist and non-specialist, urban and rural, anesthesia, surgery, and maternity work force into high-functioning networks based on the best available evidence. REPORT: Surgical and maternity triage needs to be embedded within networks to address the tensions between sustainable regional programs and local access to care. Safety and quality must be demonstrated to be equivalent across similar patients and procedures, regardless of network site. Triage of patients across multiple sites is a quality outcome metric requiring continuous iterative scrutiny. Clinical coaching between rural and regional centres can be helpful in building and sustaining high-functioning networks. Maintenance of quality and the provision of continuing professional development in low-volume settings represent a mutual value proposition. CONCLUSION: The trusting relationships that are foundational to successful networks are built through clinical coaching, continuing professional development, and quality improvement. Currently, a collaborative effort in British Columbia is delivering a provincial program-Rural Surgical Obstetrical Networks-built on the principles and supporting evidence described in this consensus statement.

Role of sildenafil in a neonate with double outlet left ventricle and pulmonary hypertension.

Double outlet left ventricle (DOLV) is a rare congenital heart defect where the aorta and the pulmonary trunk arise predominantly from the morphologic left ventricle. The clinical manifestations depend upon the location of the ventricular septal defect in relation to the great arteries, degree of pulmonary and aortic outflow tract obstruction, and other associated cardiac defects. We describe a neonate with DOLV, sub-aortic ventricular septal defect, and side-by-side great vessels who continued to require oxygen for desaturation to 60% in the neonatal period. His clinical symptoms were suggestive of persistent pulmonary hypertension, with 5-8% higher saturations in the leg compared to arm. He was started on oral sildenafil and his oxygen requirement decreased from 2 L/min to 0.1 L/min within 24 hours of initiating sildenafil. .

Clinico-histopathological correlation of symptomatic left main coronary artery electrocardiographic ischemia pattern in a young child with hypertrophic cardiomyopathy.

The specific electrocardiographic (ECG) pattern of left main coronary artery ischemia is exceptionally rare in children and under recognized. Occasional reports are bereft of a detailed mechanistic ECG description and limited to dissection, spasm, or anomalous origin of the left main coronary artery. An association with pediatric hypertrophic cardiomyopathy (HCM) is hitherto unreported. We furbish a detailed electrocardiographic description of this entity in the unusual setting of a 4-year-old child with HCM with a restrictive phenotype and exertional symptoms consistent with myocardial ischemia. Heart transplant was performed for this rare indication. Histopathology of the explanted heart in particular revealed left main coronary artery narrowing and provided us with a unique window of opportunity to correlate with clinical findings. The pathophysiology was also likely exacerbated by diffuse sub endocardial ischemia in the milieu of left ventricular hypertrophy with elevated end diastolic pressures. We anticipate that this illustrative case will help contextualize this as well as pediatric scenarios in which the 'left main ischemia' ECG pattern can be encountered and foster an accurate electrocardiographic recognition of this entity in children.

Directive clinique no 422a : Ménopause : symptômes vasomoteurs, agents thérapeutiques d'ordonnance, médecines douces et complémentaires, nutrition et mode de vie.

OBJECTIF: Proposer des stratégies pour améliorer les soins aux femmes ménopausées ou en périménopause d'après les plus récentes données probantes publiées. POPULATION CIBLE: Femmes ménopausées ou en périménopause. BéNéFICES, RISQUES ET COûTS: La population cible bénéficiera des plus récentes données scientifiques publiées que leur communiqueront les fournisseurs de soins de santé. Aucun coût ni préjudice ne sont associés à cette information, car les femmes seront libres de choisir parmi les différentes options thérapeutiques offertes pour la prise en charge des symptômes et morbidités associés à la ménopause, y compris l'abstention thérapeutique. DONNéES PROBANTES: Les auteurs ont interrogé les bases de données PubMed, Medline et Cochrane Library pour extraire des articles publiés entre 2002 et 2020 en utilisant des termes MeSH spécifiques à chacun des sujets abordés dans les 7 chapitres. MéTHODES DE VALIDATION: Les auteurs ont évalué la qualité des données probantes et la force des recommandations en utilisant le cadre méthodologique d'évaluation, de développement et d'évaluation (GRADE). Voir l'annexe A en ligne (tableau A1 pour les définitions et tableau A2 pour l'interprétation des recommandations fortes et faibles). PROFESSIONNELS CONCERNéS: médecins, y compris gynécologues, obstétriciens, médecins de famille, internistes, urgentologues; infirmières, y compris infirmières autorisées et infirmières praticiennes; pharmaciens; stagiaires, y compris étudiants en médecine, résidents, moniteurs cliniques; et autres fournisseurs de soins auprès de la population cible. DÉCLARATIONS SOMMAIRES: RECOMMANDATIONS.

Guideline No. 422a: Menopause: Vasomotor Symptoms, Prescription Therapeutic Agents, Complementary and Alternative Medicine, Nutrition, and Lifestyle.

OBJECTIVE: Provide strategies for improving the care of perimenopausal and postmenopausal women based on the most recent published evidence. TARGET POPULATION: Perimenopausal and postmenopausal women. BENEFITS, HARMS, AND COSTS: Target population will benefit from the most recent published scientific evidence provided via the information from their health care provider. No harms or costs are involved with this information since women will have the opportunity to choose among the different therapeutic options for the management of the symptoms and morbidities associated with menopause, including the option to choose no treatment. EVIDENCE: Databases consulted were PubMed, MEDLINE, and the Cochrane Library for the years 2002-2020, and MeSH search terms were specific for each topic developed through the 7 chapters. VALIDATION METHODS: The authors rated the quality of evidence and strength of recommendations using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. See online Appendix A (Tables A1 for definitions and A2 for interpretations of strong and weak recommendations). INTENDED AUDIENCE: physicians, including gynaecologists, obstetricians, family physicians, internists, emergency medicine specialists; nurses, including registered nurses and nurse practitioners; pharmacists; medical trainees, including medical students, residents, fellows; and other providers of health care for the target population. SUMMARY STATEMENTS: RECOMMENDATIONS.

Echocardiographic Indicators Associated with Adverse Clinical Course and Cardiac Sequelae in Multisystem Inflammatory Syndrome in Children with Coronavirus Disease 2019.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 causes significant cardiovascular involvement, which can be a determinant of clinical course and outcome. The aim of this study was to investigate whether echocardiographic measures of ventricular function were independently associated with adverse clinical course and cardiac sequelae in patients with MIS-C. METHODS: In a longitudinal observational study of 54 patients with MIS-C (mean age, 6.8 ± 4.4 years; 46% male; 56% African American), measures of ventricular function and morphometry at initial presentation, predischarge, and at a median of 3- and 10-week follow-up were retrospectively analyzed and were compared with those in 108 age- and gender-matched normal control subjects. The magnitude of strain is expressed as an absolute value. Risk stratification for adverse clinical course and outcomes were analyzed among the tertiles of clinical and echocardiographic data using analysis of variance and univariate and multivariate regression. RESULTS: Median left ventricular apical four-chamber peak longitudinal strain (LVA4LS) and left ventricular global longitudinal strain (LVGLS) at initial presentation were significantly decreased in patients with MIS-C compared with the normal cohort (16.2% and 15.1% vs 22.3% and 22.0%, respectively, P < .01). Patients in the lowest LVA4LS tertile (<13%) had significantly higher C-reactive protein and high-sensitivity troponin, need for intensive care, and need for mechanical life support as well as longer hospital length of stay compared with those in the highest tertile (>18.5%; P < .01). Initial LVA4LS and LVGLS were normal in 13 of 54 and 10 of 39 patients, respectively. There was no mortality. In multivariate regression, only LVA4LS was associated with both the need for intensive care and length of stay. At median 10-week follow-up to date, seven of 36 patients (19%) and six of 25 patients (24%) had abnormal LVA4LS and LVGLS, respectively. Initial LVA4LS < 16.2% indicated abnormal LVA4LS at follow-up with 100% sensitivity. CONCLUSION: Impaired LVGLS and LVA4LS at initial presentation independently indicate a higher risk for adverse acute clinical course and persistent subclinical left ventricular dysfunction at 10-week follow-up, suggesting that they could be applied to identify higher risk children with MIS-C.

Crohn's disease with dilated cardiomyopathy in a child: An experience with heart transplantation.

BACKGROUND: DCM has rarely been reported in children with CD, but is a recognized complication in adults. DCM with uncontrolled heart failure may need heart transplantation, which could be challenging in CD. CASE DESCRIPTION: We present a 9-year-old female with CD who was diagnosed with DCM and a measured Ejection fraction ~25.8%. She was initiated on heart failure treatment, but continued to have progressive symptoms and underwent bicaval OHT three months after her initial presentation. Her post-operative course was complicated by perforation of the rectosigmoid junction requiring exploratory laparotomy and primary repair of the perforation. This was believed to be secondary to her underlying CD and possibly reperfusion injury after heart transplant. During the first-year post heart transplant, she had 4 episodes of acute cellular rejection. These episodes were treated with high dose steroids with good results. The patient has been in remission from CD since transplantation. For the last two years she is doing well, with no further rejection episodes on current immunosuppression. CONCLUSION: DCM, though rare, can occur in patients with CD, whether active or in remission. The development of DCM could be insidious and keeping a high index of suspicion is important and subtle signs suggestive of cardiac involvement should warrant further investigation. The post-transplant management can be challenging with early morbidity in the first post-transplant year but overall good median survival and quality of life for our patient to date.

Pneumocystis pneumonia with hypogammaglobulinemia in a pediatric patient who underwent heart transplantation.

A 23-month-old Caucasian female who had heart transplantation (HT) at 11 days of age was diagnosed with Pneumocystis pneumonia (PCP) in the setting of secondary hypogammaglobulinemia (HGG). She was diagnosed with HGG at 5 months of age and had been receiving monthly intravenous immunoglobulin infusion. This is the first case report describing the clinical course of PCP in a pediatric patient with HGG. She developed PCP 23 months after HT even when she was off steroids and was receiving timely IVIG. The case posed some clinical questions regarding PCP prophylaxis and HGG management.

Database Autopsy: An Efficient and Effective Confidential Enquiry into Maternal Deaths in Canada.

BACKGROUND: Maternal death surveillance in Canada relies on hospitalization data, which lacks information on the underlying cause of death. We developed a method for identifying underlying causes of maternal death, and quantified the frequency of maternal death by cause. METHODS: We used data from the Discharge Abstract Database for fiscal years 2013 to 2017 to identify women who died in Canadian hospitals (excluding Quebec) while pregnant or within 1 year of the end of pregnancy. A sequential narrative based on hospital admission(s) during and after pregnancy was constituted and reviewed to assign the underlying cause of death (based on the World Health Organization's framework). Maternal deaths (i.e., while pregnant or within 42 days after the end of pregnancy) and late maternal deaths (i.e., more than 42 days to a year after the end of pregnancy) were examined separately. RESULTS: We identified 85 maternal deaths. Direct obstetric causes included 8 deaths (9%) related to complications of spontaneous or induced abortion; 9 (11%), to hypertensive disorders of pregnancy; 15 (18%), to obstetric hemorrhage; 11 (13%), to pregnancy-related infection; 16 (19%), to other obstetric complications; and <5 (<6%), to complications of management. There were 21 (25%) maternal deaths with indirect obstetric causes, and <5 (<6%) with undetermined causes. Of 120 late maternal deaths, 16 (13%) had direct obstetric causes, among them, 9 deaths by suicide (56%). One hundred late maternal deaths (83%) had indirect obstetric causes; and <5 (<4%) had undetermined causes. CONCLUSIONS: The majority of maternal deaths in Canada have direct obstetric causes, whereas most late maternal deaths have indirect obstetric causes. Suicide is an important direct cause of late maternal death.

Revisiting the Care Pathway for Trial of Labour After Cesarean: The Decision-to-Delivery Interval Is Key.

Centres providing maternity care and offering a trial of labour after cesarean must develop and use maternal educational and consent processes that emphasize choice and autonomy related to options for and decisions surrounding vaginal birth after cesarean and elective repeat cesarean delivery. These centres should have administrative systems and processes that take into account local resources for cesarean delivery services, including team-based complex maternity risk support and an urgency consensus on the fetal, maternal, and maternal-fetal indications for a surgical delivery to ensure an appropriate decision-to-delivery interval.

Incidence and Risk Factors for Acute Kidney Injury in Severely Injured Patients Using Current Kidney Disease: Improving Global Outcomes Definitions.

BACKGROUND: Acute kidney injury (AKI) is a significant cause of morbidity and mortality for critically injured trauma patients. The Kidney Disease: Improving Global Outcomes (KDIGO) practice guideline is the most up-to-date classification for AKI. The aims of this study were to determine the incidence and risk factors for AKI in critically injured trauma patients using the current KDIGO definitions. STUDY DESIGN: A prospective cohort study was performed at our academic, level 1 trauma center, from September 2017 to August 2018. All adult trauma patients admitted to the surgical ICU were included. The primary outcome was the development of AKI, as defined by KDIGO. Secondary outcomes included hospital and ICU length of stay, ventilator days, and mortality. RESULTS: There were 466 patients included and 314 (67%) developed AKI. Those who developed AKI were more often hypotensive on admission (7% vs 2%), had higher Injury Severity Scores (ISS) (19 vs 13), were more likely to have severe injuries to the chest (40% vs 24%) and extremities (20% vs 6%), received transfusion (41% vs 21%), sustained crush injuries (8% vs 1%), received radiocontrast (75% vs 47%), nephrotoxic medication (74% vs 60%), or vasopressors (15% vs 3%). After multivariate analysis, risk factors independently associated with AKI include age, Injury Severity Score (ISS), severe extremity injuries, radiocontrast, and vasopressors. Those who developed AKI had higher mortality (9% vs 2%). CONCLUSIONS: Using current KDIGO criteria, the incidence of AKI in critically injured trauma patients was higher than previously reported. Older patients, with more severe injuries to their extremities and chest and who have suffered crush injuries, appear to be the most a risk. AKI in the critically injured patient results in an almost 5-fold increase in mortality.

Stigma as a Barrier to Care for Human Papillomavirus: A Commentary Summarizing Literature and Presenting Recommendations for Health Care Providers.

In this commentary, we briefly summarize knowledge on stigma associated with human papillomavirus (HPV). In addition, we provide suggestions for health care providers to de-stigmatize HPV and improve the delivery of care.

Modeling the Balance of Benefits and Harms of Cervical Cancer Screening with Cytology and Human Papillomavirus Testing.

BACKGROUND: Benefits of screening should outweigh its potential harms. We compared various metrics to assess the balance of benefits and harms of cervical cancer screening. METHODS: We used a cervical cancer natural history Markov model calibrated to the Canadian context to simulate 100,000 unvaccinated women over a lifetime of screening with either cytology every 3 years or human papillomavirus (HPV) testing every 5 years. We estimated the balance of benefits and harms attributable to screening using various metrics, including colposcopies/life-year gained, and net lifetime quality-adjusted life-years (QALY) gained, a measure integrating women's health preferences. We present the average (minimum-maximum) model predictions. RESULTS: Cytology-based screening led to 1,319,854 screening tests, 30,395 colposcopies, 13,504 life-years gained over a lifetime, 98 screening tests/life-year gained, 2.3 (1.6-3.3) colposcopies/life-year gained, and a net lifetime gain of 10,735 QALY (5,040-17,797). HPV-based screening with cytology triage in the same population would lead to 698,250 screening tests, 73,296 colposcopies, 15,066 life-years gained over a lifetime, 46 screening tests/life-year gained, 4.9 colposcopies/life-year gained (2.9-11.1), and a net lifetime gain of 11,690 QALY (4,409-18,742). HPV-based screening was predicted to prevent more cancers, but also incur more screening harms than cytology-based screening. CONCLUSIONS: Metrics using colposcopies as the main harm outcome favored cytology-based screening, whereas metrics based on screening tests and health preferences tended to favor HPV-based screening strategies. IMPACT: Whether HPV-based screening will improve the balance between benefits and harms of cervical cancer screening depends on how the balance between benefits and harms is assessed.