10-year-old male presented with intermittent fever, cough, shortness of breath, and weight loss. Contrast CT chest performed showed pulmonary thrombus in the right lower lobe branch with necrotizing cavitary pneumonia of the right lower lobe. Dilation of the left coronary artery (LCA) was noted on the chest CT and hence dedicated CT coronary angiography was performed. On CT coronary angiography, there was dilation of the proximal LCA with a wide caliber fistula arising from the LCA, proximal to its bifurcation. The fistula coursed in a retroaortic manner, draining into the right atrium posteriorly at the superior cava-atrial junction. Blood culture showed HACEK organism which is known to be associated with endocarditis. The echocardiogram showed possible vegetation attached to the left side of the atrial septum. After the patient was medically treated with intravenous antibiotics, successful ligation of the fistula was performed at its insertion to the right atrium. We will discuss in detail as well as illustrate the CT coronary angiography findings in this patient using MIP and 3D reconstructions. We will also describe the types of coronary cameral fistula in detail Read More

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

Authors: Maller Vijetha, Maller Vinod

Keywords: coronary cameral fistula

Biliary atresia is a progressive, fibro-obliterative disease of the intra- and extrahepatic bile ducts in infancy. This exhibit outlines the pathophysiology, diagnostic pathways, and current and emerging management strategies for biliary atresia in the pediatric age group. Read More

Meeting name: SPR 2023 Annual Meeting & Postgraduate Course , 2023

Authors: Chauhan Ankita, Maller Vijetha

Keywords: Biliary Atresia, Kasai, HIDA

An isolated anomalous systemic arterial supply to a segment of the lung without associated sequestration is a rare congenital pulmonary abnormality. This anomaly was initially believed to be a type of pulmonary sequestration (Pryce type 1). However, it was later found to have different characteristics than a true sequestration. The affected pulmonary segment is supplied by an aberrant systemic artery, however, there is no abnormality of lung parenchyma such as a mass or cystic change to suggest sequestration. In addition, there is a normal pulmonary venous drainage into the left atrium and a normal bronchial tree. However, there is a fistulous communication between the abdominal aortic branch supplying the lung segment and the respective pulmonary vein which can cause an elevated systemic-to-pulmonary flow ratio due to left-to-left shunting. Sade and associates introduced a sequestration spectrum for the classification of various complexes of bronchovascular anomalies. Clements and Warner presented this anomaly as a “pulmonary malinosculation spectrum.” Patients with this condition are usually asymptomatic. They may present with hemoptysis and exertional dyspnea with a continuous heart murmur. A large shunt can lead to left-sided overload and congestive heart failure. On chest radiographs, the appearance of a tubular opacity from the upper abdomen to the right lower lobe (abdominal aortic supply to the lung) may mimic the appearance seen in Scimitar syndrome (a type of partial anomalous pulmonary venous return with hypoplasia of the right lung, which is drained by an anomalous pulmonary vein into the systemic venous system). CT angiography chest is required to make an accurate diagnosis to distinguish this anomaly from Scimitar syndrome as well as exclude the possibility of sequestration, as management is different in these conditions. In this educational poster, we will review the clinical presentation, describe the imaging findings of anomalous systemic arterial supply to normal lung using examples from our own institution, and discuss the treatment options as well as the natural progression of the disease. Recognition of the imaging features in anomalous systemic arterial supply to normal segments of the lung is important to identify patients at risk of developing left-to-left shunt. Endovascular coil embolization or surgical ligation of the aberrant systemic artery to the lung segment is usually the treatment of choice in symptomatic patients. Read More

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

Authors: Patel Chinky, Maller Vijetha

Keywords: malinosculation, sequestration, bronchovascular

Transseptal course is defined as the anomalous course of left coronary artery (LCA) after its anomalous origin from the opposite sinus, when the coronary artery takes a sharp turn to dip into the basal interventricular septum behind the right ventricular outflow tract (RVOT) and emerge laterally to the epicardial surface. Transseptal course is generally considered by some as benign entity. However, case reports of severe cardiac symptoms such as myocardial ischemia, arrhythmia, and even sudden cardiac death have been sporadically reported. Surgical management in symptomatic patients can be challenging. One of the latest surgical approach to this anomaly is transection of the RVOT, unroofing the septal course of the LCA, followed repair of the posterior wall of the RVOT by placement of rectangular strip of autologous pericardium. We present a 17-year-old male who was noted to have an anomalous left coronary artery on screening echocardiography. CT coronary angiography revealed anomalous origin of left anterior descending artery (LAD) from the right sinus of Valsalva with a long intraconal/transseptal course that measured 43mm in length. The left circumflex artery (LCx) also had anomalous origin from the right sinus of Valsalva with retroaortic course and a fistula with pulmonary artery. This patient had 5 ostia at the right sinus of Valsalva with separate origins of two conus arteries. This patient had successful transconal unroofing of the anomalous LAD and ligation of the LCX to pulmonary artery fistula. We will discuss the preoperative as well as postoperative CT coronary angiography using multiplanar views, 3D hollow reconstructions, as well as virtual endoluminal views. The CT coronary angiography characteristics of transeptal course of anomalous coronary artery will be discussed in detail. Illustrations with 3D reconstructions which helped our cardiothoracic surgeons to plan the transconal unroofing of the anomalous LAD will be discussed. Recognition of transeptal course of the coronary artery and describing its course and relation to the RVOT including the assessment of the overlying conal septal myocardial thickness with the help of multiplanar views and 3D views can help better surgical planning. Read More

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

Authors: Maller Vijetha, Boston Umar, Knott-craig Christopher

Keywords: Transseptal course, coronary fistula, transconal unroofing

A newborn male child with antenatal diagnosis of left facial mass presumably hemangioma, presented for postnatal ultrasound. Ultrasound of the left face and neck, showed a large homogenous mass measuring 5x7x4 cm arising from the inferior aspect of the superficial lobe and entire deep lobe of the parotid gland extending over the angle of mandible to the left parapharyngeal space. On MRI the mass was heterogenously enhancing with low signal intensity on T1 and intermediate signal on T2 weighted imaging. The mass was also extending into the left masticator, sublingual, and submandibular spaces. The neonate also had a right upper abdominal swelling, and an ultrasound abdomen detected heterogeneously hypo to hyperechoic mass in the segment 4 of liver. On triple phase computed tomography, the mass showed early peripheral arterial enhancement and heterogeneous enhancement in portal and venous phase. On magnetic resonance imaging, the mass was hyperintense to liver on T2-weigthed imaging with restricted diffusion and heterogenous enhancement less than the background liver on all phases. Biopsy result of the left facial mass was sialoblastoma and that of liver was hepatoblastoma. Alpha fetoprotein was also elevated. Wide margin surgical resection of sialoblastoma and left lobe hepatectomy was performed followed by chemotherapy. Sialoblastoma is a rare congenital tumor of the salivary glands, mainly arising from the parotid gland, which is locally aggressive and potentially malignant. A total of approximately 40 cases of congenital sialoblastoma has been reported. Neonatal hepatoblastomas comprises 10% of pediatric hepatoblastomas. An association of sialoblastoma and centromere division has been reported, which in turn is associated with embryonal tumors. There have been only two reported cases of concomitant sialoblastoma and hepatoblastoma. Further research with genetic analysis is required to document association of these two embryonal tumors. The current management of these tumors is complete surgical resection followed by chemotherapy. Read More

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

Authors: Maller Vijetha, Arora Anurag, Boulden Thomas, Choudhri Asim

Keywords: Sialoblastoma, Hepatoblastoma