Case Report: A 14 year-old previously healthy male presented to the emergency department with weakness, fever, diarrhea, lateral right eye deviation and transient vision loss, following a week of headaches and dizziness. The symptoms began following a recent camping trip to Yosemite National Park with friends, where distant contact with squirrels and several bug bites were noted. The initial physical examination revealed additional photophobia, limited neck flexion secondary to pain, and a raised, non-tender soft tissue mass over the anterior right shin. Soon after admission, the patient developed pain in the right hip and left leg and significant lower extremity weakness. A chest X-ray demonstrated left lower lobe consolidation, a left upper lobe lung nodule, and a right upper lobe lung nodule. MRI showed multifocal osteomyelitis and multiple intraosseous, intramuscular and soft tissue abscesses. Contrast-enhanced CT angiogram demonstrated septic emboli, scarring, and atelectasis within the left lower lung with a small hydropneumothorax. Lucent lesions were also noticed in the T3-T6 vertebral bodies with increased prominence of the paravertebral soft tissue. A lumbar puncture was found consistent with bacterial meningitis. A blood culture, CSF culture, and culture of abscess fluid from the right hip were all found to be PCR positive for <i>Yersinia pestis,</i> and the patient was placed on a treatment regimen of ciprofloxacin, gentamicin, and tetracycline. Discussion: Plague, colloquial known as the Black Death, is a zoonosis caused by the gram-negative bacterium <i>Yersinia pestis </i>and endemic to the Western United States and developing countries. In the modern day, plague is typically found in infected rodents and spread to humans through fleas. Three forms of plague are reported in humans: bubonic, septicemic, and pneumonic. Our patient’s presented primary septicemic plague complicated by secondary pneumonia, meningitis, and osteomyelitis. Notably, Yersinia osteomyelitis has been reported only once in the historical literature, and was likely underappreciated before the advent of MRI. This case presentation will highlight the unique imaging manifestations of the patient’s disseminated disease. Read More

Meeting name: IPR 2016 Conjoint Meeting & Exhibition , 2016

Authors: Jen Aaron, Iskander Paul, Ghahremani Shahnaz

Keywords: plague, osteomyelitis, pneumonia, meningitis, sepsis

Awareness of abdominal aorta abnormalities in pediatric patients is essential for appropriate diagnosis and management of the patients suffering from these conditions. Due to a nonspecific presentation, and sometimes being asymptomatic earlier in life, imaging has an important role in diagnosis of these abnormalities. Aneurysm and dissection are excluded, as they are not specific to children and with few exceptions mostly present in older patients. We review the clinical presentation and show imaging findings of mid aortic syndrome, Takayusu arteritis, rare entities such as idiopathic infantile arterial calcification. Also we show imaging of the anatomic variants of the distal aorta such as middle sacral artery, persistent sciatic artery, abdominal aorta coarctation and blind ending aorta, with a short review of embryologic development of abdominal aorta. These anomalies are not common, however if remain undiagnosed might have serious consequences. -Midaortic syndrome is an uncommon disease with progressive narrowing of the abdominal aorta and its major branches, typically involving interrenal segment of the aorta; it affects mostly children and young adults, the cause is not clear, might be the result of an intrauterine insult to the intima and subintimal tissues. Aside from diagnosis, imaging has a major role in endovascular treatment of mid aortic syndrome. -Takayusu arteritis, also predominantly involves aorta and its major branches of younger patients, with strong female predominance, and frequently found in Asian patients. Destruction of arterial medi leads to aneurysm formation and uncommonly rupture of the involved artery. -Idiopathic infantile arterial calcification, a rare entity presenting with extensive calcification and stenosis of large and medium sized arteries, usually leads to early death from coronary artery occlusion. -The aortoiliac variants are rare and not commonly discussed in the imaging literature. Some anomalies might be asymptomatic in young ages, but might complicate surgeries like heart, renal transplant, or hip surgery and increases the risk of morbidity and mortality. Some of these anomalies such as persistent sciatic artery need long term follow-up, given the possibility of aneurysmal degeneration. Read More

Meeting name: SPR 2018 Annual Meeting & Postgraduate Course , 2018

Authors: Saadat Vandad, Chawla Soni, Chen Johnathan, Iskander Paul, Ghahremani Shahnaz

Keywords: Aorta, takayusu, midaortic

Orthotopic heart transplant (OHT) is the treatment of choice for patients with end-stage heart disease including pediatric patients. Imaging has an essential role in evaluation of the perioperative and postoperative heart transplant patients. Although some of the imaging is performed and interpreted by cardiologists, a substantial portion of images are read by radiologist, therefore radiologists must be familiar with common normal and abnormal posttreatment imaging features. Many end stage heart failure patients require circulatory support as a bridge to transplant, with a ventricular assist device or intra-aortic balloon pump. The ventricular assist devices specially increase the risk of infection, and intra-aortic balloon pump requires frequent radiographic monitoring to ensure appropriate placement in the proximal descending aorta, just distal to the left subclavian artery. Proximal placement may result in great vessel compromise and distal placement may cause occlusion of mesenteric and/or renal vasculature. Expected postoperative findings in the first few weeks after surgery included enlarged cardiac silhouette, small pneumomediastinum, pneumothorax, pneumopericardium, subcutaneous emphysema, small atelectasis, and mediastinal widening. Transplant-related complications are divide to: Early complications (0-30 days): pulmonary infection, interstitial and/or alveolar pulmonary edema, and allograft failure, also symptomatic pneumothorax, mediastinal hematoma, mediastinal infection, sternal dehiscence and vascular complications. Intermediate term complications (1-12 months): acute antibody related allograft rejection, acute cellular rejection (T-cell mediated response) which is the most common form of rejection, also tricuspid regurgitation, constrictive pericarditis, drug toxicity can happen in this time period. Late postoperative complications (&gt;12 months): posttransplant lymphoproliferative disease, cardiac allograft vasculopathy, infection, kaposi sarcoma and other malignancies, aortic dissection, pseudoaneurysm formation, and thromboembolism. Read More

Meeting name: SPR 2020 Annual Meeting & Postgraduate Course , 2020

Authors: Saadat Vandad, Loo Jerry, Chen Johnathan, Iskander Paul, Chawla Soni, Ghahremani Shahnaz

Keywords: OHT, Complications, Imaging

Intestinal failure is a rare but devastating condition in pediatric patients that is often caused by short bowel syndrome (SBS) secondary to necrotizing enterocolitis, volvulus, or intestinal atresia. Patients require the use of total parenteral nutrition (TPN) to survive, which can lead to cholestasis and end stage liver disease (ESLD). Treatment of intestinal failure in pediatric patients who have developed subsequent liver failure from TPN with multivisceral (MVTx) transplantation has progressed dramatically in the past two decades as a result of improvements in immunosuppression, surgical technique, and post-transplant care. The number of transplantations has always been relatively low due to a lack of appropriate donors and the rarity of intestinal failure. However, given the high acuity of patients that do ultimately undergo these procedures, recognition of post-operative complications is critical for graft survival and improved outcomes. This educational exhibit will review the indications and relevant anatomy for these types of transplantations. Magnetic resonance (MR) findings related to post-transplantation complications will be presented with imaging correlates from other modalities (CT, PET/CT, US) as needed. Read More

Meeting name: SPR 2017 Annual Meeting & Categorical Course , 2017

Authors: Loo Jerry, Iskander Paul, Chen Johnathan, Hall Theodore, Boechat Ines, Ghahremani Shahnaz

Keywords: intestinal, liver, magnetic resonance

We developed a free iOS application, RADiDOC, with the goal of simplifying the complex subject of medical radiation risk for pediatric patients and their parents. Utilization of medical imaging has risen over the past decade, making the topic of radiation safety an increasingly important issue that is increasingly featured in the popular media. Furthermore, given the unique risk profile of radiation exposure in the pediatric age group, there has been increased focus on the judicious use of ionizing radiation in the imaging of children. These efforts have resulted in the Image Gently campaign, which has offered an immense contribution to pediatric radiation safety issues. In keeping with these efforts, we created the RADiDOC application to help pediatric patients and their parents understand key radiation safety information in a simple, intuitive way. To meet these ends, RADiDOC was created with three core functionality modes: (1) Consultation, (2) Radiation Log, and (3) Radiation 101. Consultation mode allows users to explore a multitude of imaging studies and image-guided procedures and learn about their associated radiation risk, including quantitative cancer risk estimated using the BIER VII dataset. To be more meaningful to patients, dose is described not only in terms of “effective dose” and its associated ACR category, but also in terms of intuitive measures such as the equivalent number of transatlantic flights and days of natural sun exposure. The Radiation Log mode allows patients or their parents to log additive radiation risk from all imaging received in their lifetime, creating a longitudinal radiation log of lifetime exposure. This may be of particular interest to pediatric oncology patients who receive long-term serial imaging. Finally, Radiation 101 offers a primer on the basics of radiation safety and radiation physics aimed at a broad audience of laypeople. RADiDOC offers a free resource to facilitate the discussion of radiation risk between pediatric radiologists, clinicians, pediatric patients, and their parents. In this Technical Development presentation, we review key radiation safety concepts and elaborate on the core functionalities of this application and how it can be of use to pediatrics radiologists and their patients. Read More

Meeting name: SPR 2017 Annual Meeting & Categorical Course , 2017

Authors: Seals Kevin, Tesoriero Joseph, Cagnon Christopher, Boechat Ines, Ghahremani Shahnaz, Iskander Paul

Keywords: Radiation, Radiation Dose, Radiation Safety

Scimitar syndrome, also called congenital venolobar syndrome, is a rare phenomenon characterized by partial anomalous pulmonary venous return (PAPVR) with variable degrees of right lung and pulmonary artery hypoplasia. In this report, we present an extremely rare case demonstrating co-occurrence of Tetralogy of Fallot (TOF) and scimitar syndrome. A two-day-old female was presented with dyspnea and acidosis. An echocardiography and subsequent ECG-gated CT angiogram of the chest revealed findings of TOF and complete pulmonary atresia, accompanied by arterial and ventricular septal defects (ASD, VSD) and a large patent ductus arteriosus (PDA) connecting to hypoplastic left pulmonary artery. Additionally, PAPVR was observed with drainage from the hypoplastic right lung to the IVC in the form of scimitar syndrome. Correctional surgery was undertaken, with creation of a right ventricular to pulmonary artery conduit, pulmonary arterio-plasty, ASD closure and creation of a fenestrated VSD patch. Follow-up imaging in two years showed development of multiple aortopulmonary collateral arteries (MAPCAs), persistent PAPVR and development of chronic right lung interstitial edema. When there is suspicion for the association of TOF and scimitar syndrome, CT angiography, gated cardiac MRI, and 3-D contrast enhanced MR angiography are all preferred diagnostic modalities, providing excellent delineation of the abnormal pulmonary vein, its course and drainage, and quantification of the pulmonary venous flow. The anomalous vein most commonly drains into the IVC and less likely to the hepatic veins, right atrium, or portal vein. While scimitar syndorme in conjunction with Tetralogy of Fallot is an extremely rare association, meticulous evaluation of the pulmonary venous connections is important in all patients with TOF, as the presence of PAPVR can result in right ventricular dilation and dysfunction. Read More

Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024

Authors: Kooraki Soheil, Iskander Paul, Ghahremani Shahnaz, Prosper Ashley, Finn John, Bedayat Arash

Keywords: Scimitar, Tetralogy Of Fallot, PAPVR