Congenital focal lung malformations (CLM) are a heterogeneous group of lesions that have been commonly classified by their features and the presence of systemic vascular supply into bronchial atresia, congenital pulmonary airway malformation, bronchogenic cyst and bronchopulmonary sequestration, however, pathologically there is considerable overlap not recognized by this classification method. Literature has hypothesized that a common pathogenetic origin of developmental bronchial obstruction, variable in timing and extent, is responsible for the spectrum observed. The purpose of this case report is to describe the findings of an unusual CLM with concurrent intra and extrapulmonary lesions demonstrated by pre and postnatal imaging, gross and microscopic pathology. A 19 year-old G1P0 female presented at 34 weeks gestation for evaluation of a fetal chest mass, first identified by US at 32 weeks. Fetal US showed a large right thoracic lung lesion containing two distinct macro cysts; fetal MR demonstrated T2 hyperintense signal throughout the lesion and increased volume of the affected lung with resultant mild mediastinal shift. No systemic feeding vessel was evident. The baby was born at 39 weeks and required intubation due to respiratory distress. Postnatal CTA showed a solid lesion right thoracic apex outlined by pleural air with systemic arterial supply from the subclavian artery, no visible airways, and a central cyst with peripheral atelectasis. There was an additional unilocular cyst with air-fluid level in the perihilar right middle lung. Thoracotomy on day 4 described an extrapulmonary sequestration (EPS) right thoracic apex and cystic lesions in the right upper and middle lobes, all of which were resected without complication. Pathology revealed similar histology throughout all resected tissue characterized by markedly dilated proximal bronchi and parenchymal maldevelopment with immature alveolar spaces as is seen in bronchial atresia, in addition to diffuse pulmonary interstitial glycogenosis. This case of a CLM with concurrent solid and cystic lesions supports a common pathogenetic mechanism based on developmental bronchial obstruction both with and without retention of embryonic systemic vascular communication. Prenatal classification into distinct lesions is hampered by overlapping pathological features. Characterization of vascular and airway connections and awareness of potential complications are most important to guide counseling and therapy. Read More

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

Authors: Stein Jill, Browne Lorna

Keywords: CPAM, Sequestration, Bronchial Atresia

Vascular tumors are commonly encountered in infants and children, the vast majority being infantile hemangiomas with less frequent congenital hemangiomas. Less commonly encountered vascular tumors are challenging due to their rarity. However, these often have distinguishing clinical, imaging, and histologic features which enable diagnosis and dictate management. The objective of this electronic exhibit is to present the clinical and imaging findings of vascular tumors encountered in a large children’s hospital over a 10-year period. These will be grouped according to the ISSVA benign, locally-aggressive, and malignant classification of vascular tumors. We will include unusual presentations of infantile and congenital hemangiomas, as well as the clinical features and imaging appearances of the less commonly encountered vascular tumors (tufted angioma, spindle cell hemangioma, pyogenic granuloma, epithelioid hemangioma, kaposiform hemangioendothelioma, and angiosarcoma). Treatment options and prognosis will also be discussed. Read More

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

Authors: Harrison Andrew, Browne Lorna

Keywords: angiosarcoma, hemangioma, ISSVA

Background: Vitamin C deficiency remains an underrecognized cause of musculoskeletal symptoms in children with suboptimal nutrition. Vitamin C plays major roles in collagen, nitric oxide, and norepinephrine formation, and is essential from the diet because humans cannot synthesize it. In children with scurvy, musculoskeletal symptoms such as limp or refusal to bear weight, occur in approximately 80% of patients. Early recognition can prevent invasive, costly workups and limit morbidity. Objective: To characterize skeletal imaging features of scurvy in a pediatric case series. Read More

Meeting name: SPR 2022 Annual Meeting & Postgraduate Course , 2022

Authors: Pryor William, Browne Lorna, Gilley Stephanie, Moore Jaime, Fenton Laura

Keywords: scurvy, vitamin C deficiency, MRI

Infants with one functional cardiac ventricle often undergo the Norwood procedure which seeks to create unobstructed outflow from the single ventricle to aorta using a complex aortic arch reconstruction. The distal region of the patch used to fashion the neo-aorta terminates near the aortic isthmus, and represents an area of extensive challenge and manipulation for cardiothoracic surgeons. Re-coarctation in this region is common after the Norwood procedure and may be linked with longer-term morbidity and mortality. Echocardiography (TTE) is the primary means of surveillance of the aortic isthmus, though measurements are often difficult due to poor acoustic windows and lack of patient cooperation. To date, there have been no studies that assess how accurately TTE measures the aortic isthmus relative to a computed tomography (CT) derived gold standard. Read More

Meeting name: IPR 2016 Conjoint Meeting & Exhibition , 2016

Authors: Ehrmann Daniel, Dimaria Michael, Fonseca Brian, Younoszai Adel, Browne Lorna

Keywords: Congenital Heart Disease, Single ventricle, Aortic Isthmus, Norwood, Coarctation

Infants with one functional cardiac ventricle undergo operations that yield unobstructed systemic outflow and a controlled source of pulmonary blood flow, often starting with the Norwood operation with Blalock-Taussig (BT) shunt. Infants with shunt dependent pulmonary blood flow are at risk for developing proximal pulmonary artery (PA) stenoses, which may result in morbidity and mortality. Echocardiography (TTE) is the primary means of surveillance for PA narrowing, but is limited by acoustic windows, operator experience and patient cooperation. Computed tomography (CT) offers high spatial resolution, rapid acquisition and relatively low radiation exposure. To date, there have been no studies comparing TTE and CT-derived measurements of the proximal PA anatomy in this high-risk population. Read More

Meeting name: IPR 2016 Conjoint Meeting & Exhibition , 2016

Authors: Ehrmann Daniel, Browne Lorna, Fonseca Brian, Younoszai Adel, Dimaria Michael

Keywords: Congenital Heart Disease, Single ventricle, Pulmonary Artery, CT, Echocardiography

The advent of fast CT scanners with lower radiation doses has resulted in rapid growth in the numbers of cardiac CTs being performed in children. This has also increased the number of CTs interpreted by cardiologists, similar to adult cardiac CT. Pediatric cardiac CT demonstrates multiple potential extracardiac variants and pathology that may occur in conjunction with congenital heart disease (CHD). Prior publications in adult cohorts demonstrate a wide-range and incidence of extracardiac findings (6-39%) but the prevalence in children is unknown. Therefore, the purpose of this abstract is to describe incidence, distribution and significance of the extracardiac findings found in pediatric cardiac CT at a tertiary referral center. Read More

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

Authors: Sassoon Daniel, Malone Ladonna, Weinman Jason, Mcgraw Marty, Barker Alex, Browne Lorna

Keywords: cardiac, extracardiac

4D flow MRI with full coverage of the heart and proximal vessels is a powerful tool to understand hemodynamic pathology arising from congenital heart disease (CHD) and subsequent surgery; however, scan times often exceed 10 minutes thereby limiting widespread adoption in pediatrics. Decreasing scan time using compressed-SENSE (CS), a combination of conventional parallel imaging (SENSE) and randomized spatial undersampling, shows promise over SENSE alone. However, both the feasibility and its effect on accurate flow quantification in pediatrics has not been evaluated. This study investigated the feasibility of CS for pediatric 4D flow MRI to quantify flow indices used in risk stratification of CHD such as coarctation or pulmonary regurgitation, in comparison with SENSE. Read More

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

Authors: Fujiwara Takashi, Browne Lorna, Malone Ladonna, Lu Quin, Fonseca Brian, Dimaria Michael, Barker Alex

Keywords: 4D flow, Compressed SENSE, Congenital heart disease

A 22-year-old G2P1 female presented to our institution at 22 weeks for fetal cardiology evaluation of hypoplastic left heart syndrome (HLHS) with mitral stenosis and aortic atresia. Fetal echocardiogram confirmed the diagnosis and suggested a severely restricted atrial septum with a pulmonary venous forward/reverse time-velocity integral ratio (VTI) of 2.7. At 29 weeks' gestation, she underwent a fetal CMR to evaluate for pulmonary lymphangiectasia and interatrial septum. Fetal cardiac gating was obtained with an MR-compatible Doppler ultrasound device (Smartsync, Northh Medical) and imaging was performed at 3T. In addition to the findings of HLHS, findings at fetal CMR included an intact/severely restrictive atrial septum with markedly dilated pulmonary veins. Additionally, there was an anomalous pulmonary vein connecting the right sided pulmonary veins to both the azygos vein and the SVC. 4D flow MRI quantified the distribution of blood flow, demonstrated a similar pulmonary venous forward/reverse flow ratio of 2.4 and visualized the anomalous vessel decompressing the pulmonary venous system into the systemic venous system. There was a segmental pattern of pulmonary lymphangiectasia, present in the right middle lobe and lingula. Same day fetal echocardiography also confirmed the anatomical findings. The patient was determined not to be a candidate for fetal intervention and following counselling, the family elected for comfort care at delivery. After delivery, the baby did not demonstrate clinical signs of a restrictive atrial septum, despite an atrial septal gradient of 11mmHg by echocardiography, and following re-evaluation, underwent a staged single ventricle palliation. At surgery for stage 1 (Norwood-Sano), the atrial septum was found to be almost intact. The infant continues to do well and currently is status post stage 2 with a superior cavopulmonary anastomosis. This case demonstrates the use of fetal CMR with an MR compatible doppler ultrasound device in the fetal evaluation of HLHS with a suspected restrictive atrial septum. In this case, fetal CMR with 4D flow was able to calculate a pulmonary venous forward/reverse flow ratio similar to echocardiography and demonstrated the anomalous pulmonary venous drainage pathway, which likely accounted for the absence of the expected severe manifestations of an intact atrial septum in addition to the relatively mild manifestations of pulmonary lymphangiectasia. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Fuentealba Cargill Andrea, Friesen Richard, Barker Alex, Fujiwara Takashi, Englund Erin, Park Sungho, Londono Obregon Camila, Browne Lorna

Keywords: 4D Flow, Pulmonary Venous Return, Cardiac MRI

Previous studies show that phase contrast MRI (PC-MRI) can successfully measure fetal blood flow in late gestation singleton fetuses. To date there is a paucity of data quantifying fetal blood flow of twin or early gestational age pregnancies (~19 weeks). Twin-twin transfusion syndrome (TTTS) is a rare complication of monochorionic pregnancies that is believed to result from inequal flow distribution between fetuses. In this novel investigation, we utilized PC-MRI to investigate fetal aortic flow in early gestational age fetuses with TTTS or selective Intrauterine Growth Restriction (sIUGR). We compared flow distribution between the smaller and larger fetus in each twin pair. Read More

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

Authors: Barhaghi Krystle, Schuchardt Eleanor, Schafer Michal, Meyers Mariana, Behrendt Nicholas, Barker Alex, Cuneo Bettina, Friesen Richard, Browne Lorna

Keywords: Fetal MRI, Twins, Phase Contrast MRI

A 29-year-old female (G6P4) was referred to our center following identification of thoracoomphalopagus conjoined twins, with fused heart and liver, at 20 weeks of gestation. Fetal echocardiogram and fetal cardiac MR (CMR) were performed at 21 weeks gestation to evaluate potential for post-natal repair. Fetal CMR was performed at 1.5 Tesla using an MRI compatible fetal doppler ultrasound (DUS) device (smart-sync, Northh imaging) for cardiac gating. Protocol included multiplanar cine SSFP sequences and multiplanar blackblood sequences. Fetal echocardiography and CMR were used together to understand the complex anatomical arrangements. Each twin had 2 sets of atria (with an interatrial connection) but only a single ventricle. Twin A had a left ventricle, pulmonary atresia and MAPCAs. Twin B had a right ventricle had coarctation of the aorta, bilateral SVCs and partial anomalous pulmonary venous return. After counselling regarding the grave prognosis of both twins, the patient decided to deliver at her local hospital with comfort care. Teaching Points from this Case This case of conjoined twins at 21 weeks gestation, is the youngest example of successful clinical DUS fetal CMR. In this case, fetal CMR was used to demonstrate the great vessel anatomy and was fundamental in ascertaining the absence of a path to successful surgical palliation for either twin. Additionally, fetal CMR was used as the primary modality during patient counselling to explain the anomalies. The high degree of diagnostic certainty that the fetal CMR facilitated, allowed the family to avoid the unnecessary emotional and financial expense that relocation for delivery would have incurred and facilitated palliative care at their local center. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Fuentealba Cargill Andrea, Friesen Richard, Fujiwara Takashi, Park Sungho, Arguello Fletes Gladys, Englund Erin, Barker Alex, Londono Obregon Camila, Browne Lorna

Keywords: Fetal MRI, Cardiac MRI, 4D Flow

<b>Introduction</b>: Conventional pediatric MRI acquisitions of a short axis (SA) stack for ventricular volumes typically requires one breath-hold per slice, resulting in multiple coordinated breath-holds under anesthesia to acquire the entire stack. Here, we aim to validate a compressed sensing approach to reduce breath-holding during SA balanced steady state free precession (bSSFP) cine imaging. Equivalency is tested via qualitative image scoring and global volumes compared to conventional bSSFP cine imaging. Read More

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

Authors: Naresh Nivedita, Browne Lorna, Barker Alex, Malone Ladonna, Fujiwara Takashi, Hulseberg-dwyer Emma, Mcgee Janet, Lu Quin, Twite Mark, Dimaria Michael, Fonseca Brian

Keywords: Compressed Sensing, Cardiac Function, Anesthesia