Pediatric Patient with a Diagnosis of Pelvic Extraosseous Ewing's Sarcoma: A Case Report.

Ewing's sarcoma (ES) is a malignant tumor that arises mainly from bone tissue. Primary extraosseous Ewing sarcoma (EES) is a rare form of the Ewing's sarcoma family of tumor, and pelvic localization is even more unusual, considered to be one of the rarest localizations [1]. We present the case of a seven-year-old boy with persistent abdominal pain. Ultrasound (US), contrast-enhanced computed tomography (CECT), and magnetic resonance imaging (MRI) revealed the presence of a large, solid, and heterogeneous mass in the pelvis. The histological and immunohistochemistry were compatible with pelvic EES. Teaching point: Extraosseous Ewing's sarcoma is a rare pediatric tumoral entity that requires clinician and radiological vigilance and detection.

Case Report: Challenges in the etiology of left ventricular aneurysm.

Left ventricular aneurysms are outpouchings delineated by a thin myocardial wall, more frequently encountered at the apex of the left ventricle, which is seldom dyskinetic or akinetic. Apart from coronary artery disease, the etiology can be challenging. We report the case of a 30-year-old man with an isolated apical left ventricular aneurysm associated with prominent trabeculations on echocardiography.

Paediatric presentation of cardiac involvement in hypereosinophilic syndrome.

Eosinophilic endomyocarditis is extremely rare in paediatrics. This case report aims to present the sequential changes from thrombotic to fibrotic stages of the cardiac involvement in a child with hypereosinophilic syndrome. Endomyocardial fibrosis progressively aggravated the severity of the mitral regurgitation. Bioprosthetic valve replacement was finally performed without recurrence of cardiac complications despite a late relapse of the underlying disease.

Long-term conservative management of a giant cardiac fibroma.

A giant cardiac fibroma was discovered during evaluation for a soft systolic murmur in an asymptomatic 2-week-old girl. Echocardiography and magnetic resonance imaging showed a large intraventricular solid mass developed at the expense of the left ventricular lateral wall. Tumour progression resulted in failure to thrive and ventricular arrhythmia between 2 and 18 months of age. At that time, complete resection seemed unfeasible and conservative management with heart failure and antiarrhythmic medications was chosen. All drugs were discontinued when the patient was 5 years old. Since that time, the mass is stable and the patient is strictly asymptomatic. Conservative strategy seems to be acceptable in selected cases but close follow-up is mandatory.

An atypical evolution of tako-tsubo cardiomyopathy.

An 83-year-old woman with a possible history of myocardial infarction presented with acute chest pain and dyspnoea. The electrocardiogram showed no signs of acute coronary syndrome, but Q waves in the anteroseptal leads. The blood tests showed an important elevation of troponin T level. Emergency coronary angiography revealed normal epicardial arteries, but the left ventriculogram demonstrated anterior akinesis and ballooning with normal apical and basal function. An atypical takotsubo cardiomyopathy was suspected and the patient received beta blocker and ACE inhibitor treatment. Two days later the echocardiogram evolved to a classical pattern of apical ballooning with normalization on the follow-up examination 5 weeks from the acute episode. To our knowledge, this is the first case report describing an evolving pattern of wall motion abnormality from an anterior wall ballooning to an apical form.

The impact of hydration on renal measurements and on cortical echogenicity in children.

BACKGROUND: Kidney ultrasonography is frequently performed in children; to date there are no specific guidelines regarding hydration before the examination. OBJECTIVE: Because certain renal measurements can be indicative of pathology, we used sequential US images to investigate the effect of standardised hydration in children relative to renal size, echogenicity and pelvic expansion. MATERIALS AND METHODS: Thirty-one children (7 years to 14 years old) underwent US examinations before hydration (T0) and at 30 min (T30) and 60 min (T60) after ingesting water. We measured bladder volume, inter-polar kidney size, renal volume and anterior-posterior diameter of the pelvis. Cortical echogenicity was compared to that of the liver and spleen. RESULTS: On the right side the increase in average inter-polar renal size was 2.5% at T30 and 2.6% at T60 (P < 0.05) compared to T0. On the left the increase was 3.8% at T30 and 4.3% at T60 (P < 0.05). Volume expansion for the right kidney was 16.8% at T30 and 14.0% at T60 (P < 0.05). On the left it was 12.9% at T30 and 10.2% at T60 (P < 0.05). Cortical echogenicity progressed from hypoechogenicity to isoechogenicity (P < 0.05). The percentage of expanded pelves increased with hydration (T0: 3.2%, T30: 9.7%, T60: 22.6%). CONCLUSION: Oral hydration influences renal length, volume and echogenicity in children. Hydration results in expansion of the pelvis. We recommend standardisation of hydration before US examination.

Extensive peritoneal uptake of technetium-99m-labelled hydroxy diphosphonate in a patient undergoing hemodialysis.

A 48-year-old African lady started peritoneal dialysis in 1994 after transplant failure. After several episodes of peritonitis and hemorrhagic ascites, hemodialysis was resumed in 2003 because of small bowel subocclusion. In 2006, an abdominal CT showed calcifications and dilatation of intestinal loops. Sclerosing peritonitis was diagnosed and she underwent enterolysis and resection of the peritoneal capsule. She remained asymptomatic until 2009 when she presented again with subocclusion. Because of generalized skeletal pain, a bone scan was performed, which showed Tc-99m hydroxy diphosphonate uptake on the peritoneum. The patient is currently on hemodialysis with parenteral nutrition.

Ten-year experience with surgical treatment of adults with congenital cardiac disease.

The number of adults with congenital cardiac disease continues to increase, and adult patients are now more numerous than paediatric patients. We sought to identify risk factors for perioperative death and report our results with surgical management of adult patients with congenital cardiac disease. We retrospectively analysed in-hospital data for 244 consecutive adult patients who underwent surgical treatment of congenital cardiac disease in our centre between January, 1998 and December, 2007. The mean patient age was 27.2 plus or minus 11.9 years, 29% were in functional class III or IV, and 25% were cyanosed. Of the patients, half were operated on for the first time. A total of 61% of patients underwent curative operations, 36% a reoperation after curative treatment, and 3% a palliative operation. Overall mortality was 4.9%. Predictive factors for hospital death were functional class, cyanosis, non-sinus rhythm, a history of only palliative previous operation(s), and an indication for palliative treatment. Functional class, cyanosis, type of initial congenital cardiac disease (single ventricle and double-outlet right ventricle), and only palliative previous operation were risk factors for prolonged intensive care stay (more than 48 hours). The surgical management of adult patients with congenital cardiac disease has improved during recent decades. These generally young patients, with a complex pathology, today present a low post-operative morbidity and mortality. Patients having undergone palliative surgery and reaching adulthood without curative treatment present with an increased risk of morbidity and mortality. Univentricular hearts and double-outlet right ventricles were associated with the highest morbidity.

Plasma osteoprotegerin is an independent risk factor for mortality and an early biomarker of coronary vascular calcification in chronic kidney disease.

BACKGROUND: Cardiovascular disease is the major cause of morbidity and mortality in chronic kidney disease (CKD) and early biomarkers are required which can predict disease and death in such patients. The aim of our study was to investigate if osteoprotegerin (OPG) could be a predictor of coronary artery calcification (CAC) and mortality in CKD. METHODS: A total of 77 outpatients (32 with pre-dialysis CKD and 45 undergoing hemodialysis) were followed-up during 2 years. Measurements of CAC were performed using Siemens Multidetector CT software and calcium scores were measured according to the Agatston method. RESULTS: OPG was an independent predictor of the Agatston score for CAC and correlated with the degree of CAC in pre-dialysis patients. A two-sample t-test characterized survivors as having a better glomerular filtration rate, lower Agatston scores, and lower serum levels of OPG. Kaplan-Meier survival curves separated survivors from non-survivors at plasma OPG cut-off levels of <3.1 ng/mL. A multivariable logistic regression analysis showed that OPG was an independent predictor of mortality from all causes in CKD patients. CONCLUSIONS: OPG predicted mortality in CKD patients and could be a valuable biomarker in early detection of CAC in these patients.

Fatal cerebritis and brain abscesses following a nontraumatic subdural hematoma in a chronic hemodialyzed patient.

Staphylococcus aureus is the leading cause of bacteremia in hemodialysis-dependent patients that can lead to metastatic abscesses with poor outcome. We report a case of a 65-year-old chronic hemodialyzed male patient who developed cerebritis and brain abscesses complicating a spontaneous subdural hematoma, following Staphylococcus aureus bacteremia related to infected arteriovenous fistula. In spite of adequate antibiotherapy and several surgical brain drainages, our patient did not survive. Prevention of S. aureus is highly important in hemodialysis patients.