Paraduodenal hernias are the most common type of congenital internal hernia. Because of its overall rare incidence, this entity is often overlooked during initial assessment of the patient. Lack of specific diagnostic criteria also makes diagnosis exceedingly difficult, and the resulting diagnostic delays can lead to tragic outcomes for patients. Despite these perceived barriers to timely diagnosis, there may be specific radiographic findings that, when combined with the appropriate constellation of clinical symptoms, would aid in diagnosis. This patient first presented at 8 years of age with vague symptoms of postprandial emesis, chronic abdominal pain, nausea, and syncope. Over the span of 6 years he was evaluated 2 to 3 times a year with similar complaints, all of which quickly resolved spontaneously. He underwent multiple laboratory, imaging, and endoscopic studies, which were nondiagnostic. It was not until he developed signs of a high-grade obstruction and extremis that he was found to have a large left paraduodenal hernia that had volvulized around the superior mesenteric axis. This resulted in the loss of the entire superior mesenteric axis distribution of the small and large intestine and necrosis of the duodenum. In cases of chronic intermittent obstruction without clear etiology, careful attention and consideration should be given to the constellation of symptoms, imaging studies, and potential use of diagnostic laparoscopy. Increased vigilance by primary care and consulting physicians is necessary to detect this rare but readily correctable condition.
Papillary fibroelastomas are benign primary cardiac tumors that usually arise from the valve apparatus and are rare in the pediatric population. Involvement of the tricuspid valve is even less common with only a few cases reported in the literature. Cardiac magnetic resonance imaging is a valuable examination that aids in differentiating a tumor from a thrombus. We present the case of an 11-month-old girl referred by her pediatrician to investigate a murmur noted since birth. To our knowledge, this is the first report of a pathologically proven papillary fibroelastoma arising from the tricuspid valve characterized by magnetic resonance imaging in an infant.
Background: Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death (SCD) in children. Surgical intervention is recommended for AAOLCA with an interarterial course, with other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of AAOLCA in a prospective cohort following a standardized approach. Methods: All patients with AAOLCA <20 years old were prospectively enrolled. Anatomic details were obtained with coronary computerized tomography angiography (CTA). Myocardial functional studies were obtained under provocative stress, including exercise stress test, stress nuclear perfusion imaging, or dobutamine stress cardiac magnetic resonance. Patients were stratified as high-risk if: 1) episode of aborted SCD, 2) interarterial and/or intramural course, or 3) evidence of myocardial ischemia on functional studies. Results: A total of 47 patients (32 male, 68%) with AAOLCA were enrolled, median age 12 [IQR 6.0;15.0] years. Of these, 20 had exertional symptoms (42.5%) and 5 had aborted SCD (10.5%). Thirty-one patients (79%) were classified high-risk, 28% low-risk, and 3 (6%) work-up is ongoing. Of patients with completed work-up, 16/43 (37%) had inducible ischemia and, of these, 2/16 (13%) presented without symptoms and one with aborted SCD as the first symptom. Two patients in our cohort had AAOLCA arising close to the noncoronary sinus, just above the left and noncoronary commissure, resulting in ostial stenosis: one presented with aborted SCD and another with left ventricular dysfunction. To date, 17 patients have undergone repair, median age 10 years [IQR 6.6;15.7]. Median follow-up was 3.9 [IQR 1.3;6.3] years with no significant symptoms and all but 4 patients are cleared from exercise restrictions. Conclusion: AAOLCA may present with diverse anatomic configuration and risk stratification is essential to determine management. AAOLCA from the noncoronary sinus may also present with myocardial ischemia and, thus, be classified as high-risk. A multimodality imaging approach is necessary to adequately risk stratify this population. All patients are alive at last follow-up and the majority free from exercise restriction.
The Coronavirus Disease 2019 (COVID-19) pandemic has led to an exponential rise in mental health issues. Studies have shown that, in times of increased unemployment rates and economic downturn, rates of mental health issues, suicide, substance use, and domestic violence tend to increase. Barriers to care, including stigma and decreased access to providers, contribute to morbidity and mortality. Telehealth services are being utilized to help increase access to care, and economic stimulus packages have been created to help with the financial burden that is often associated with increased mental health stressors. Efforts to prevent burnout and other policy recommendations can help decrease mental health issues in first responders and health care professionals, who are at an increased risk for these problems. Increasing the ability to provide wellness screenings to the general population, to educate the public about preventive measures and practices, and to provide mental health and substance use treatment, such as medication management and therapy services, are among top priorities to further reduce the socioeconomic impact of COVID-19 on mental illness.
Methods to identify children with cystic fibrosis (CF) at risk for development of advanced liver disease are lacking. We aim to determine the association between liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) with research ultrasound (US) patterns and conventional hepatic markers as a potential means to follow liver disease progression in children with CF. ELASTIC (Longitudinal Assessment of Transient Elastography in CF) is a nested cohort of 141 patients, ages 7-21, enrolled in the Prediction by US of Risk of Hepatic Cirrhosis in CF (PUSH) Study. We studied the association between LSM with research-grade US patterns (normal [NL], heterogeneous [HTG], homogeneous [HMG], or nodular [NOD]) and conventional hepatic markers. In a subgroup (n = 79), the association between controlled attenuation parameter (CAP) and US pattern was explored. Among 133 subjects undergoing VCTE, NOD participants (n = 26) had a significantly higher median (interquartile range) LSM of 9.1 kPa (6.3, 15.8) versus NL (n = 72, 5.1 kPa [4.2, 7.0]; P < 0.0001), HMG (n = 17, 5.9 kPa [5.2, 7.8]; P = 0.0013), and HTG (n = 18, 6.1 kPa [4.7, 7.0]; P = 0.0008) participants. HMG participants (n = 14) had a significantly higher mean CAP (SD) (270.5 dB/m [61.1]) compared with NL (n = 40, 218.8 dB/m [46.5]; P = 0.0027), HTG (n = 10, 218.1 dB/m [60.7]; P = 0.044), and NOD (n = 15, 222.7 dB/m [56.4]; P = 0.041) participants. LSM had a negative correlation with platelet count (rs = - 0.28, P = 0.0071) and positive correlation with aspartate aminotransferase-to-platelet ratio index (rs = 0.38, P = 0.0002), Fibrosis-4 index (rs = 0.36, P = 0.0007), gamma-glutamyltransferase (GGT; rs = 0.35, P = 0.0017), GGT-to-platelet ratio (rs = 0.35, P = 0.003), and US spleen size z-score (rs = 0.27, P = 0.0073). Conclusion: VCTE is associated with US patterns and conventional markers in patients with liver disease with CF.
