A 15 year old female with no significant past medical history except for long standing dysphagia and intermittent chest pain presented for a frontal and lateral radiograph. The x-ray showed a long segment density along the right heart border concerning for mediastinal mass. The patient subsequently underwent a contrast enhanced CT. Diffuse circumferential thickening of the esophagus began just below the thoracic inlet and extending for approximately 17cm to the level of the esophageal hiatus. Evaluation of the esophageal lumen was performed at our institution utilizing reduced pediatric dose pulsed fluoroscopy with a barium esophagram. While the cervical and upper 1/3 of the thoracic esophagus had a normal lumen diameter and contour, there was irregular contractility and motility throughout the upper esophagus. The lower 2/3 showed narrowing which did distend with barium passage. At this juncture a biopsy of the lesion was performed with the resulting pathology consistent with a leiomyoma. Preoperative planning MRI was then undertaken. As seen with the CT, diffuse circumferential thickening of the esophagus began just below the thoracic inlet with progressive thickening continuing distally to a maximum thickness just above the GE junction. The patient subsequently went on to have an Ivor-Lewis esophagectomy with gastric pull through. The diagnosis of diffuse esophageal leiomyomatosis was confirmed by pathology. Diffuse esophageal leiomyomatosis (DEL) was probably first described by Hall in 1916 in a case report of a 17 year old female who died of starvation due to dysphagia, with the diagnosis subsequently made on autopsy. While some cases of DEL are sporadic, as in our case, there is a well-established association with the x-linked Alport Syndrome, especially in the pediatric population. Up to 5% of Alport patients are affected by DEL and as much as 2/3 of pediatric patients with DEL carry the diagnosis of Alport Syndrome. Esophageal-Vulvar syndrome, characterized by leiomyomata of both the vulva and esophagus, presents with findings of DEL on imaging in many cases, often in young adult females. While presentations may vary, the majority of patients present with long standing dysphagia. An Iver-Lewis esophagectomy with a gastric pull-through is the treatment of choice. Read More

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

Authors: Rapp Jordan, Mallon Mea

Keywords: Esophagus, Leiomyomatosis

To describe ultrasound (US) and magnetic resonance imaging (MRI) findings of giant hepatic regenerative nodules (GHRN) in pediatric patients with Alagille Syndrome (AS). Read More

Meeting name: IPR 2016 Conjoint Meeting & Exhibition , 2016

Authors: Rapp Jordan, Anupindi Sudha, Bellah Richard

Keywords: liver mass, cirrhosis

Background: Dual source dual-energy CT scanners (DECT) has allowed for the collection of two data sets with a single scan, opening the potential for functional data acquisition. The technique combines two energy beams at distinct voltages applied concurrently during a single scanning phase. The source data can be combined to generate a single mixed composite image, or iodine can be subtracted to create a contrast map or a virtual non-contrast image. The result is functional information in the setting of decreased radiation dose when replacing a biphasic scan, or dose neutral when compared to conventional single source CT. Post processing lung perfusion software allows for imaging display (qualification) and quantification of iodinated contrast volumes in the lungs, a surrogate for lung perfusion. Purpose: This educational exhibit will demonstrate: 1. How to perform DECT in children, 2. How to use postprocessing software, and 3. How to interpret lung perfusion results through clinical examples of current pediatric clinical indications including pulmonary embolism, lung hypoplasia, pulmonary AV malformation, and pulmonary hypertension. Conclusion: Through this exhibit, readers will gain familiarity with technical aspects of DECT of the lungs in children, understand the basics of post processing and recognize focal or regional perfusion defects, segmented perfusion analysis, and focal lesion perfusion characteristics as well as identify future applications. Read More

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

Authors: Rapp Jordan, Saul David, Kaplan Summer, Barrera Christian, Otero Hansel

Keywords: dual energy, thoracic, perfusion

The rare entity of an absent pulmonary artery has appeared in the literature since 1868, with most cases associated with congenital heart disease of various types. It has also long been observed that the absent pulmonary artery is contralateral to the aortic arch in almost every case. Isolated absence of a single pulmonary artery without associated congenital heart disease is less common, and these patients may present at any time from prenatal screening, neonatal period, early childhood, or even adolescence and adulthood. We will discuss the embryologic origins, clinical presentations, expected imaging findings, and treatment options based on patient ages from newborn to adolescence. In neonates with an isolated absent pulmonary artery, a patent ductus arteriosus will allow for continued systemic blood supply. Even early on, narrowing of the PDA may be seen as involution is inevitable without intervention. The lung parenchyma is typically preserved, without yet evidence of hypoplasia or oligemia. Once the PDA has closed, robust collateral formation will occur. As patients age without repair, the lung parenchyma may become hypoplastic with diminished lung volumes and vascular markings. Findings suggestive of recurrent infection such as bronchiectasis may also be evident. Early discovery and treatment is ideal as this will allow for prevention of long term sequelae and the greatest restoration of lung function as the options for repair are limited in the older patient. There is no universal standard approach for repairing the underlying mechanism of providing blood flow to the intrapulmonary pulmonary artery. Early intervention in neonates included PDA stenting or anastomosing the main pulmonary artery with the intrapulmonary pulmonary artery using a synthetic graft. Patients that present after the neonatal period are not likely to be eligible for surgical repair. The most common long term effect of an absent pulmonary artery is pulmonary hypertension, seen in 40% of patients. The entity of isolated unilateral absence of a pulmonary artery is rare, however demonstrates typical cardiothoracic findings depending on age at presentation. Understanding of embryology, specifically the 4th and 6th primitive aortic arches, allows one to understand why this malformation occurred and what findings to expect on imaging. The maintained PDA is vital for early lung blood supply and development and can aid in repair. Read More

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

Authors: Rapp Jordan, Poletto Erica, Urbine Jaqueline, Malik Archana, Kazmi Faaiza, Mallon Mea

Keywords: Pulmonary artery, congenital, CTA