Hypertrophic pulmonary osteoarthropathy (HPOA) is a syndrome characterized by excessive proliferation of skin and bone in the distal extremities. The classic imaging finding is symmetric, smooth periosteal reaction of the bones of the forearm or lower leg. The pathogenesis of this new bone formation is not well understood. Proposed mechanisms include growth factor release mediated by the tumor itself or the shunting of megakaryocytes through the pulmonary vasculature. These growth factors may contribute to vascular proliferation and bone formation. HPOA can be idiopathic but is more commonly secondary in patients with a variety of pulmonary disorders, congenital heart disease, and inflammatory bowel disease. HPOA is more commonly seen in adults than children. We present a case of HPOA in a patient with pleuropulmonary blastoma, a rare pediatric intrathoracic tumor. A 3 year-old girl was diagnosed with pleuropulmonary blastoma after presenting to the emergency department with a twelve-day history of fever and leg pain. As part of a fever of unknown origin workup, chest radiographs were performed, which showed a large left lower lobe mass. Contrast-enhanced computed tomography characterized the mass as mixed cystic and solid. As part of the preoperative evaluation for osseous metastatic disease, a technetium-99m bone scan was performed, which revealed bilateral, symmetric radiotracer uptake within the ulnar and fibular shafts, and the distal humeri. Subsequent radiographs of these bones demonstrated bilateral, symmetric smooth periosteal reaction. Following surgical resection of the pleuropulmonary blastoma and completion of chemotherapy, a bone scan was repeated which showed resolution of the previously seen scintigraphic findings. To date, HPOA has never been reported in a patient with pleuropulmonary blastoma. This case report highlights that HPOA, commonly thought of as an entity of adult lung disease, can also present in children. Read More

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

Authors: Poletto Erica, Fox Evan, Malik Archana, Geller Evan

Keywords: Hypertrophic Pulmonary Osteoarthropathy, Pleuropulmonary Blastoma

To describe the fluoroscopic practice patterns during pregnancy in pediatric radiologists and potential impact on professional relationships and career. Read More

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

Authors: Kim Jane, Son Jennifer, Poletto Erica, Phelps Andrew, Levin Terry

Keywords: Pregnancy, Fluoroscopy

There is a wide array of pathologies which can cause diffuse changes in the pediatric kidney. The purpose of the presentation is to show a multimodality pictorial review of characteristic findings of a wide variety of diffuse renal abnormalities in the pediatric patient. Read More

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

Authors: Goldwasser Bernard, Kazmi Faaiza, Urbine Jacqueline, Poletto Erica, Malik Archana, Mallon Mary

Keywords: renal mass, congenital renal abnormalities, syndromes affecting the kidney

When evaluating the pediatric neuroaxis, it is important to include the extra-axial spaces in the radiologists’ search pattern. The purpose of the presentation is to show a multimodality pictorial review of characteristic findings of extra-axial CNS lesions detected on CT and MRI. Read More

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

Authors: Goldwasser Bernard, Malik Archana, Poletto Erica, Urbine Jacqueline, Kazmi Faaiza, Mallon Mary

Keywords: dural mass, extra-axial masses

The formation of one or more intestinal strictures is a known complication of necrotizing enterocolitis (NEC). Multiple prior investigations have found that the occurrence of colonic strictures is higher than small intestinal strictures, at an approximate ratio of 2-3:1. We hypothesized that small intestinal strictures may be more prevalent than colonic strictures at our urban, academic, tertiary care children’s hospital. Our first aim was to determine the incidence of small intestinal and colonic strictures in patients with prior medically treated NEC who had not undergone previous surgical intervention. Second, we attempted to evaluate for differences in demographics and other clinical variables between the group of patients with small intestinal strictures versus the group with colonic strictures. Read More

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

Authors: Poletto Erica, Richards Matthew, Goldwasser Bernard, Meckmongkol Teerin, Ciullo Sean, Prasad Rajeev

Keywords: necrotizing enterocolitis, stricture

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

Hypoxic-ischemic encephalopathy is a potentially devastating neurological diagnosis for which prompt recognition is crucial for patient management. The appearance of HIE on imaging depends on the duration and severity of the hypoperfusion injury, which can range from global to subtle. As such, the differential diagnosis is wide, including infectious, metabolic, and congenital dysmyelination causes.<b> </b>It is also critical for the pediatric radiologist to be aware of possible confounding cases when presented with imaging features seen in HIE which would significantly change management. Read More

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

Authors: Misiura Anne, Urbine Jaqueline, Mallon Mea, Malik Archana, Kazmi Faaiza, Poletto Erica

Keywords: Hypoxic, Infection, Encephalopathy

The duplication of structures in the body has been a curious topic to the field of medicine for centuries. It is rare to find a radiologist who has not seen at least one duplicated or partially duplicated organ, usually of the genitourinary system, and often incidentally. While implications of GU duplication have been study previously, often due to infertility or renal issues, other organ system duplications and their implications to the patient often remain a mystery. Read More

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

Authors: Misiura Anne, Kazmi Faaiza, Poletto Erica, Mallon Mea, Malik Archana, Urbine Jaqueline

Keywords: Congenital, duplication, organs

Small round blue cell tumors (SRBCT) are a category of malignant tumors that share the characteristic histologic feature of small round undifferentiated cells. These tumors predominantly affect the pediatric population. While SRBCT are similar at the cellular level, their location, imaging characteristics, clinical manifestations, and outcomes are widely variable. The purpose of this educational exhibit is to provide a pictorial review of small round cell blue tumors and to highlight key imaging findings that will aid the radiologist when faced with such tumors. Read More

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

Authors: Yoon Susan, White Stacy, Poletto Erica, Urbine Jaqueline, Malik Archana, Mallon Mea, Kazmi Faaiza

Keywords: Small round blue cell tumors