Leonhardt Leah

Final Pr. ID: Poster #: CR-001

Cardiac strangulation is an unusual phenomenon occuring in pediatric cardiac surgical patients. During implantation of epicardial leads, a loop is made to allow for somatic growth of the heart. The mechanism proposed describes somatic growth of the pediatric heart after placement of circumferential leads, whereby they become adherent and contribute to a compressive process. This pattern has a unique radiologic appearance, and is important for radiolgists to recognize, as the entitiy has a very high mortality and morbidity, leading to ventricular compromise, valvular dysfyunction and coronary vessel compresseion.

We present two cases of cardiac strangulation by epicardial leads at our instution. The first case is a 7 year-old who presented with ventricular fibrillation and cardiac arrest, while the second was discovered following arterial switch procedure. In both cases, cardiac CTA demonstrates extrensic compression of coronary vessels, with variable ventricular deformity.

Interestingly, a majority of reported cases of strangulation occur after placement of leads at the anterior surface of the pericardium, with diaphragmatic placement appearing less frequeently as a cause. It can be inferred that this complication should be more highly considered in patients with circumferential looping over the anterior pericardium on conventional radiography, prompting more advanced imaging. Early recognition of this phenomenon is sure to prevent the highly morbid complications associated with this entity. Read More

Authors:  Leonhardt Leah

Keywords:  pacemaker ,  pacer

Maller Vijetha,  Boston Umar,  Knott-craig Christopher

Final Pr. ID: Poster #: CR-003

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

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

Keywords:  Transseptal course ,  coronary fistula ,  transconal unroofing

Maller Vijetha,  Maller Vinod

Final Pr. ID: Poster #: CR-004

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

Authors:  Maller Vijetha,  Maller Vinod

Keywords:  coronary cameral fistula

Ortega García Diana,  Rodríguez Garza Claudia,  Canales Nañez Sofìa,  Terán Helda Estrella,  Montemayor Martínez Alberto

Final Pr. ID: Poster #: CR-005

Atretic cephalocele is a rare disruption of neural tube closure. The reported incidence ranges from 1 in 3500 to 1 in 5000 live births. Is a small subscalp lesion that consist of dura, fibrous tissue, and dysplastic brain tissue. Its most common presentation it is a palpable midline parietal soft tissue mass, occasionally occipital, with a wide range of clinical presentations, the patient may have normal neurological development or have severe mental retardations, since it could be associated with multiple intracranial malformations including a fenestrated superior sagittal sinus (the most common), persistent falcine sinus, vertical embryonic positioning of the straight sinus, holoprosencephaly, hydrocephalus, eye anomalies, agenesis of the corpus callosum, among others, therefore is important to know about this condition as well as to identify its radiological characteristics using different diagnostic imaging methods to make a certain diagnostic. Transfontanellar brain ultrasound could be used as the first imaging method as it enables a non-invasive method, in addition the patients are not exposed to ionizing radiation, being the most consistent finding a subcutaneous, hypoechoic mass, with a solid fibrous tract connecting the extracranial lesion, through a small bony cranium defect, to the intracranial space; additionally in order to evaluate a further extension of the fibrous tract or the presence of other anomalies, CT or MRI can be performed, considering to the MRI as a better study because it can be used in pediatric population since there is no exposure to radiation. Main differential diagnoses of atretic cephalocele are sinus pericranii and dermoid cyst, other possible causes include haemangioma, sebaceous cyst, inclusion cyst, lipoma, tumors (Langerhans cell histiocytosis, sarcomas, etc.), bone and soft tissue infections and traumatic lesions. This educational poster pretends to review the radiological findings of two cases of patients with atretic cephalocele moreover a literature review since the published works regarding is limited and few case series or case reports have been documented. Read More

Authors:  Ortega García Diana,  Rodríguez Garza Claudia,  Canales Nañez Sofìa,  Terán Helda Estrella,  Montemayor Martínez Alberto

Keywords:  ATRETIC CEPHALOCELE ,  CYST ,  SCALP MASS

Huynh Minh-huy,  Didier Ryne,  Feygin Tamara,  Paidas Teefey Christina,  Coleman Beverly,  Oliver Edward

Final Pr. ID: Poster #: CR-006

Kaposiform hemangioendotheliomas (KHE) are rare, locally aggressive vascular neoplasms. KHE most commonly arise from the extremities and less frequently the trunk and head and neck regions. Kasabach-Merritt phenomenon complicates 70% of cases. Three cases of KHE underwent prenatal evaluation and postnatal management at our center. We report the imaging findings that may allow for improved prenatal diagnosis.

Case 1: 31-year-old G4P2 evaluated for cervicofacial mass at 24 weeks 5 days. Fetal ultrasound demonstrated a large, infiltrative, predominately solid, mildly heterogeneous mass with a volume of 62 mL. No calcifications or lesion hypervascularity were present. Same day MRI confirmed an infiltrative, predominately solid mass with mild heterogenous T2 signal and no internal flow voids. Repeat MRI at 34 weeks 5 days revealed increased lesion heterogeneity and internal flow voids. US at 36 weeks 3 days demonstrated a volume of 525 mL and lesion hypervascularity. Delivery was at 36 weeks 5 days by EXIT procedure. Kasabach-Merritt phenomenon developed, and postnatal biopsy was consistent with KHE.

Case 2: 35-year-old G2P1 evaluated for cervical mass at 40 weeks 4 days. Fetal ultrasound demonstrated a large, infiltrative predominately solid, mildly heterogeneous mass with a volume of 180 mL. Calcifications and hypervascularity were present. Fetal MRI was not performed. Delivery the next day was by cesarean section. Kasabach-Merritt phenomenon developed. Postnatal biopsy was consistent with KHE.

Case 3: 30-year-old G1P0 evaluated for cervical mass at 38 weeks 1 day. Fetal ultrasound demonstrated a large, predominately solid, heterogeneous mass with a volume of 242 mL. Calcifications and hypervascularity were present. Same day fetal MRI confirmed an infiltrative, predominately solid mass with heterogeneous T2 signal and internal flow voids. Delivery was at 38 weeks 3 days by cesarean section. Kasabach-Merritt phenomenon developed, and postnatal MRI and clinical findings were consistent with KHE. Read More

Authors:  Huynh Minh-huy,  Didier Ryne,  Feygin Tamara,  Paidas Teefey Christina,  Coleman Beverly,  Oliver Edward

Keywords:  Kaposiform hemangioendothelioma ,  cervical masses

Bhatia Anmol,  R Rajath,  Rani Usha,  Saini Shiv,  Saxena Akshay,  Sodhi Kushaljit

Final Pr. ID: Poster #: CR-007

A full-term newborn male was admitted to pediatric emergency on day one of life with respiratory distress and dysmorphism, which included radio-ulnar hemimelia, retrognathia, scoliosis, and clinodactyly. Antenatal fetal echocardiography had shown presence of mildly dilated right atrium and right ventricle with the possibility of aortic arch anomaly. Infantogram showed absent radius on right side with hypoplastic radius on left side, multiple digital anomalies in bilateral hands, and multiple segmentation anomalies in the entire spine. Echocardiography was done and it showed the presence of atrial septal defect (ASD), ventricular septal defect (VSD) and patent ductus arteriosus (PDA). However, the arch of aorta and the descending thoracic aorta could not be evaluated due to poor acoustic window.
For better delineation of arch anatomy, the child underwent Computed tomography angiography (CTA). CTA confirmed the findings of ASD and VSD. In addition, there was dextrocardia with the heart being placed in the right hemithorax. A band of pulmonary parenchyma was seen extending between the bases of right and left lung between the heart anteriorly and the esophagus and spine posteriorly, thus establishing the diagnosis of horseshoe lung. The right lung was hypoplastic and two blind ending air filled diverticulae were seen arising from trachea at D2-D3 level and D5-D6 level, possibly representing atretic/ rudimentary right-sided bronchi. The bronchus supplying hypoplastic right lung was seen to arise from the left main bronchus suggestive of a bridging bronchus, which was diffusely attenuated in caliber with near complete occlusion in its proximal part near its origin. The main pulmonary artery was dilated. The left pulmonary artery was seen to be coursing anterior to the esophagus behind the left main bronchus, thus confirming the presence of pulmonary arterial sling. The right pulmonary artery was seen arising as a small caliber vessel from the left pulmonary artery and was seen supplying the hypoplastic right lung. Apart from the above findings, there was also presence of coarctation of aorta just distal to the origin of the left subclavian artery for a length of ~6mm with post stenotic dilatation. Considering the various co-existing anomalies on the imaging, a final diagnosis of LACHT syndrome (Mardini-Nyhan association) was made. Read More

Authors:  Bhatia Anmol,  R Rajath,  Rani Usha,  Saini Shiv,  Saxena Akshay,  Sodhi Kushaljit

Keywords:  LACHT ,  Mardini-Nyhan ,  Horseshoe lung

Gupta Amit,  Naranje Priyanka,  Kandasamy Devasenathipathy,  Jana Manisha

Final Pr. ID: Poster #: CR-008

Introduction
Hepatic visceral larva migrans (VLM) is an increasingly recognized parasitic manifestation wherein migratory nematode larvae get lodged in hepatic parenchyma leading to inflammatory granuloma or abscess formation. In this report, we describe a case of VLM presenting with upper gastrointestinal (GI) bleed secondary to a rare complication, a hepatic artery pseudoaneurysm. On literature search, only one such case is reported.
Case Report
A 13-year-old girl presented with abdominal pain and progressively increasing jaundice for the last 2 weeks. The patient had multiple episodes of hematemesis and melaena requiring 2 units of blood transfusion during this time. Ultrasound (USG) abdomen showed multiple ill-defined hyperechoic lesions in the right lobe of liver with presence of anechoic cystic areas within. A dual phase Computed Tomography (CT) scan of the abdomen revealed multiple confluent and discrete hypodense lesions in the right lobe of liver more apparent on portal venous phase, along with presence of a 1.5 cm pseudoaneurysm arising from posterior branch of right hepatic artery. A differential diagnosis of pyogenic liver abscesses, hepatic VLM, disseminated tuberculosis and immunodeficiency with invasive fungal infection was kept along with hepatic artery pseudoaneurysm leading to hemobilia. Lab investigations revealed peripheral eosinophilia (54%) and raised serum IgE (1600 IU/mL). Hydatid serology was negative. Based on these findings, a diagnosis of hepatic VLM was made and the patient was started on Albendazole (400 mg BD). The patient underwent Digital Subtraction Angiography and managed with successful embolization of the pseudoaneurysm using microcoils. There was significant improvement in jaundice and no recurrence of hematemesis over the next ten days along with rapid drop in peripheral eosinophilia. The patient subsequently remained asymptomatic over a follow-up period of 18 months. The inflammation caused by cytotoxic eosinophil-derived proteins associated with helminthic parasites may lead to vascular complications.
Conclusion
Hepatic VLM can be rarely associated with hepatic artery pseudoaneurysm presenting in the form of acute upper GI bleed in the background of progressive abdominal symptoms secondary to hepatic lesions. Prompt diagnosis and management with percutaneous angio-embolization for the aneurysm and anti-helminthic therapy for the underlying parasitic infestation is of paramount importance for a good clinical outcome. Read More

Authors:  Gupta Amit,  Naranje Priyanka,  Kandasamy Devasenathipathy,  Jana Manisha

Keywords:  Visceral larva migrans ,  Pseudoaneurysm ,  GI bleed

Lakhani Dhairya,  Hailemichael Eyassu

Final Pr. ID: Poster #: CR-010

A 6-month-old full-term infant presented to the urology clinic for management of diphallia. No reported relevant past medical history. Physical examination demonstrated 2 completely developed penis with normal looking meatus, both penises covered at the mid-part of the shaft with one penile skin shaft, penoscrotal malposition, right side scrotal skin tag, sacral dimple, and undescended testes. Both of them have urine flow from the urethras. Amniotic karyotype was 46 XY. Read More

Authors:  Lakhani Dhairya,  Hailemichael Eyassu

Keywords:  Diphallia ,  Bifid phallus

England Elizabeth,  Sarma Asha,  Thomas John,  Liang Jiancong,  Snyder Elizabeth

Final Pr. ID: Poster #: CR-011

Intrascrotal lipoblastomas are rare, benign paratesticular tumors that arise from embryologic adipose tissue and most often affect young children. Due to the rare nature of these tumors, preoperative diagnosis can be challenging, despite imaging features that are somewhat distinctive with respect to more common lesions (e.g., rhabdomyosarcoma). Prospective diagnosis, however, has potential to influence the operative management strategy (for example, minimally invasive mass excision versus radical inguinal orchidectomy). Since 2016, only two cases of preoperative imaging diagnosis of scrotal lipoblastoma have been published. This case report will describe a unique case of an incidentally found intrascrotal lipoblastoma and discuss the pertinent sonographic features of this rare entity. A 3-year-old boy initially presented for evaluation for an atrophic and undescended right testicle. During that evaluation, the patient was incidentally found to have a palpable left scrotal mass. Subsequent scrotal ultrasound showed an 2.7 x 1.9 x 2.4 cm well circumscribed ovoid mass inferior to the left testicle which was in the inguinal canal. The mass appeared predominantly hypoechoic with mild internal vascularity and also contained areas of hyperechogenicity resembling fat. The ultrasound appearance of the mass was not felt to be typical of a rhabdomyosarcoma (the most common paratesticular mass in children) and other fat-containing masses, including lipoblastoma, were suggested as diagnostic considerations. Complete excision of the left paratesticular mass was successful and subsequent scrotal orchidopexy was performed. Microscopic examination of the mass demonstrated lobules of adipocytes with occasional lipoblasts. Rearrangement of the PLAG1 (8q12.1) locus was detected, confirming the diagnosis of lipoblastoma. Although rare, prospective imaging detection of intrascrotal lipoblastoma may allow for less invasive and morbid surgical intervention and decreased risk for local recurrence. Read More

Authors:  England Elizabeth,  Sarma Asha,  Thomas John,  Liang Jiancong,  Snyder Elizabeth

Keywords:  Paratesticular tumor ,  Lipoblastoma

Smith Jennifer,  Richards Allyson,  Revels Jonathan

Final Pr. ID: Poster #: CR-012

Imaging findings are diagnostic of obstructed hemi-vagina with ipsilateral renal agenesis (OHVIRA). This is a rare condition consisting of a triad of uterine didelphys (a type III Müllerian duct anomaly), hematocolpos, and ipsilateral renal agenesis. If any of these three features are absent, the diagnosis of OHVIRA is excluded. Clinical presentation is typically 2-12 months post menarche. Though rare pre-pubertal onset has been documented secondary to maternal hormonal influence, the mean age of presentation is 17 years old. Symptoms are typically non-specific including dysmenorrhea and a palpable pelvic mass.

Complications:
Acute complications of OHVIRA include primarily infection of retained blood products resulting in pyohematocolpos or pyosalpinx. Rarely pelviperitonitis can occur if hematocolpos progresses to a hematosalpinx which ruptures. Long term complications include endometriosis, thought secondary to retrograde flow of blood products, which can lead to development of pelvic adhesions. Infertility issues are thought largely to be secondary to endometriosis and/or infection more so than uterine didelphys. Additional obstetric complications are greater in patients without surgical correction and most patients who carry a pregnancy to term have no increased difficulty.

Treatment:
Surgical correction with vaginal septectomy or division is the first line treatment for OHVIRA. If surgical correction is achieved early, the risk of complications including infertility are greatly diminished. Read More

Authors:  Smith Jennifer,  Richards Allyson,  Revels Jonathan

Keywords:  OHVIRA

Emad-eldin Sally,  Taher Heba,  Osama Reham,  Gad Mostafa,  Fares Ahmed

Final Pr. ID: Poster #: CR-013

A Two-year-old female child from South Sudan presented to us with ARM and a huge perineal mass covering the whole perineum with unusual female external genitalia.
The child had previous simple loop sigmoid colostomy which was done in Sudan at day two after birth. She had an average weight percentile for age, fully active with no special features.
Formal examination of the perineum was performed under general anesthesia revealed a large perineal Lipoma extending from tip of coccyx posteriorly to a mildly separated pubic bone anteriorly. There was duplication of the introitus with two openings, each flanked by the single laterally located labia majora which are separated by the perineal mass. The clitoris was duplicated with one is seen on either side. The two orifices on either side were communicating with each other as a catheter introduced from one orifice exited through the other. The vaginal and urethral orifices could not be identified from the outside; however, there was intermittent discharge of urine from both introitus openings. The anal opening was absent. The muscle complex was examined by muscle stimulator and the point of maximal contraction was more on right side of midline.
Abdominal US showed absence of associated renal abnormalities. Genitography and distal loopogram examinations were performed in the same setting. A catheter was introduced through the right introitus, with opacification of uterine cavity and vagina, faint opacification of the rectum is noted, suggesting recto-vestibular fistula which was confirmed in distal loopogram examination.
Pelvic MRI examination showed a large fat signal intensity lesion at the perineal region with separation of the symphysis pubis. There were two vaginal introitus, with the left one reached to the uterus, the two vaginal introitus were communicating with each other through a transverse tract. Examined portion of the spine revealed dysplastic sacrum with paramidline orientation of the visualized malformed sacral segments, together with tethered cord & small intraspinal sacral lipoma (caudal regression syndrome).
Surgery for reconstruction of perineal body, external genitalia, excision of the perineal mass performed in the same sitting with anorectoplasty. Histopathological examination of the mass revealed mature fat cells.
Inconclusion ARMs may be associated with complex anomalies, proper preoperative assessment and imaging are essential before any attempt of surgical intervention. Read More

Authors:  Emad-eldin Sally,  Taher Heba,  Osama Reham,  Gad Mostafa,  Fares Ahmed

Keywords:  Duplicated clitoris ,  vestibular anus ,  perineal lipoma

Infante Juan

Final Pr. ID: Poster #: CR-014

Urinary bladder injuries are classified based on intraperitoneal versus extraperitoneal location. Intraperitoneal lacerations are associated with a higher risk of sepsis and tend to be large and are therefore treated with prompt surgical exploration and repair. In contrast, extraperitoneal bladder ruptures are commonly managed conservatively via simple catheter drainage with healing commonly achieved between 10 days and 3 weeks. However, lower urinary tract injuries are an exception to the rule which also require emergent intervention.

The subject of this case report is an 8-year old girl that suffered lacerations to the bladder neck, bladder trigone, and pelvic floor during a motor vehicle collision. The initial contrast-enhanced phase demonstrated bladder wall thickening and intraluminal blood clot. Bladder rupture was not directly apparent until the patient was called back for a delayed scan of the pelvis which showed contrast extravasation from the trigone pooling around retroperitoneal spaces including the mesorectal fascia surrounding the rectum and the presacral space. Although these are not intraperitoneal locations, the radiologist should be aware that lacerations involving the bladder neck and lower urinary tract warrant immediate surgical consultation.

Lower urinary tract injuries are sufficiently uncommon that an experienced urologist may encounter only a handful during their career. This case report highlights the anatomic spaces in the pelvis that are necessary to troubleshoot the likely location of a bladder injury and that the lack of intraperitoneal pooling of contrast should not distract from the emergent nature of this rare injury. The common association of lower urinary tract injury with pelvic floor disruption is also highlighted in this case as the patient had lacerations and pooling of contrast extending along the vagina and into the labia. Finally, the importance of delayed excretory phase imaging in the setting of pelvic trauma is emphasized to allow for the prompt diagnosis of bladder trauma. Read More

Authors:  Infante Juan

Keywords:  trauma ,  bladder ,  pelvic anatomy

Collins Lee,  Cohen Sara,  Pomeranz Christy,  Jarrett Delma,  Baad Michael,  Akhavan Ardavan,  Kovanlikaya Arzu

Final Pr. ID: Poster #: CR-016

Congenital urethral polyps are a rare benign fibroepithelial lesion, typically arising from the verumontanum. We present a case of a 2 day old neonate undergoing renal ultrasound for follow up of prenatally diagnosed urinary tract dilation. Ultrasound demonstrated severe bilateral central and peripheral caliyectasis with parenchymal thinning, bilateral distal ureterectasis and marked bladder wall thickening. An echogenic soft tissue nodule was visualized at the bladder outlet. Subsequent VCUG showed a rounded filling defect intermittently located in the posterior urethra and bladder neck, suspicious for a diving mobile polyp. Transurethral resection of the polyp was performed. This case highlights an unusual cause of bladder outlet obstruction in neonates which can mimic the presentation and imaging appearance of posterior urethral valves. Read More

Authors:  Collins Lee,  Cohen Sara,  Pomeranz Christy,  Jarrett Delma,  Baad Michael,  Akhavan Ardavan,  Kovanlikaya Arzu

Keywords:  congenital urethral polyp ,  urinary tract dilation

Markovitz Michael,  Hemmrich Megan,  Francis Christopher

Final Pr. ID: Poster #: CR-017

Cystic teratoma and lymphatic malformation (LM) are two generally benign differential considerations for congenital masses which demonstrate overlapping clinical and imaging findings. It is important to distinguish between these etiologies as cystic teratomas may require surgical excision whereas lymphatic malformations may be treated with minimally invasive sclerotherapy. We present two contrasting cases: a cervical neck teratoma mimicking a LM and an abdominal LM resembling a cystic teratoma.

Case 1: A 9-month-old female presented with a right craniofacial neck mass discovered on prenatal screening. Postnatal ultrasound demonstrated a complex multiloculated cystic lesion with peripheral vascularity. MRI confirmed a predominantly cystic, mixed soft tissue and cystic right anterolateral neck mass, most consistent with mixed type lymphatic malformation. Despite two rounds of percutaneous sclerotherapy with doxycycline, the lesion continued to enlarge on repeat MRI, crossing the midline and causing mass effect on the trachea with associated airway compromise. Respiratory distress was exacerbated by COVID-19 infection requiring intubation and eventual tracheostomy. She was started on sirolimus and underwent a third sclerotherapy treatment with doxycycline and STS without improvement. Surgical debulking was performed with pathology returning as mature multicystic teratoma composed of endodermal, neuroglial and mesodermal elements with minimal fat.

Case 2: A 13-year-old male with no significant past medical history presented with three weeks of right-sided abdominal pain and constipation. CT showed a right abdominal multiseptated cystic lesion. MRI confirmed a 27 cm multicystic abdominal mass with small soft tissue component and calcification with mass effect on the small bowel and right ureter causing partial small bowel obstruction and hydronephrosis, most consistent with a large cystic teratoma. However, following surgical excision pathology demonstrated a multicystic mass containing vascular structures with lymphocytic aggregates and nodules consistent with LM.

Expanding on these cases, this exhibit will also review the clinical presentations and management of cystic teratoma and LM with an emphasis on imaging workup and radiology-pathology correlation. It is important for radiologists and their interdisciplinary colleagues to be cognizant of the overlapping features of these conditions to avoid errors in diagnosis and management as their treatments differ. Read More

Authors:  Markovitz Michael,  Hemmrich Megan,  Francis Christopher

Keywords:  Lymphatic Malformation ,  Teratoma ,  Cystic

Jayapal Praveen,  Alharthi Omar,  Thakor Avnesh

Final Pr. ID: Poster #: CR-018

HISTORY AND CLINICAL COURSE:A 16-year-old male presented with worsening abdominal pain, non-bloody diarrhea, and unintentional weight loss. On exam, he had diffuse abdominal tenderness and unnoticed left testicular swelling. US demonstrated a heterogeneous left testicular mass; CT demonstrated a large heterogeneous left testicular mass with extensive tumor thrombus propagating along the left gonadal vein, into the renal vein, the IVC, right atrium, right ventricle with several metastatic pulmonary emboli. The patient underwent left orchiectomy, and pathology showed testicular mixed germ cell tumor with immature teratoma and yolk sac components. The patient was referred to our institution for further management of his intravascular and intracardiac tumor components, given the patient’s pathology fell under an intermediate category based on the “Intermediate Germ Cell Consensus classification” which supports treatment with curative intent given an 80% long-term survival rate.
PROCEDURE:An open surgical approach was used to remove tumor tissue from the right heart and supra-hepatic IVC. Percutaneous endovascular thrombectomy was then used to remove the tumor from the infra-hepatic IVC and the left renal vein 24 hours after the cardiac surgery.
Step 1: Distal Embolic Control: A single disc from an Inari Flow Triever device was deployed, via the right internal jugular vein, below the level of the hepatic veins to provide distal embolic control throughout the procedure. This is an alternative application for this device.
Step2: IVC tumor removal: An Inari Clot Triever device was deployed via the right common femoral vein. Multiple 360-degree sweeps throughout the IVC to ensure intravascular tumor removal as validated on follow-up IVC venography.
Step 3: Left renal vein tumor removal: The left renal vein thrombus was removed using a combination of the Flow Triever and aspiration catheter systems. IVUS and left renal venogram confirmed the tumor removal.
FOLLOW-UP:The hospital course was uneventful. The patient was anticoagulated with a tight INR goal of 1.5-2 to balance the need for anticoagulation and avoiding the risk of bleeding from recent cardiac surgery. Chemotherapy with Bleomycin, Cisplatin, and Etoposide was initiated upon discharge and will be reviewed by surgical oncology for retroperitoneal nodal clearance later. This case demonstrates the multi-disciplinary team approach to caring for a complex case of an extensive testicular tumor in a pediatric patient. Read More

Authors:  Jayapal Praveen,  Alharthi Omar,  Thakor Avnesh

Keywords:  Endovascular ,  thrombectomy ,  testicular tumor

Regmi Pradeep,  Amatya Isha

Final Pr. ID: Poster #: CR-019

Primary tubercular osteomyelitis accounts for less than 10% of extra-pulmonary tuberculosis (TB) in childhooduMultifocal tuberculosis with more than one site especially in immune-competent children is rare. Very few cases of multifocal involvement of ribs and sternum are reported. We are presenting a case of a 14-year-old boy presented with painless swelling in the chest.
Clinical history
The 4-year-old child presented with painless swelling on his anterior chest in midline and back pain on and off for 3 months duration. No history of trauma. However, he lost about 3 kilograms of his weight in this duration. On examination, the swelling was soft and painless on palpation. No exit points in the skin. There was no lymphadenopathy. However, other systemic examinations were unremarkable. Lab parameters revealed a WBC count of 11,000 with lymphocyte-predominant.
In children, the bones most commonly affected are the metaphysis of the long bones (femur, tibia, fibula, radius, ulna and humerus), spine, pelvic girdle and skull bones. Multifocal skeletal tuberculosis is uncommon in children and accounts for less than 10% of skeletal tuberculosis. Sternum TB commonly occurs by reactivation of latent focuses formed throughout the course of hematogenous or lymphatic dissemination of primary tuberculosis. Extra-pulmonary tuberculosis in children varies from 21-44%. Pericardial involvement is one of the crucial sites of involvement. About 10% of children develop constrictive pericarditis in follow up for pericardial effusion.
The diagnosis of bone tuberculosis is particularly challenging as the presentation of skeletal tuberculosis is often insidious, with an absence of the classical features of pulmonary tuberculosis or typical systemic features such as fever, cough, night sweats or weight loss (history of localized painless swelling and weight loss were present in our case). The gold standard for diagnosis comprises histological examination verification. Needle aspiration or excisional biopsy for histopathological diagnosis of sternum TB is obligatory. Both Needle aspiration and biopsy were performed in our case.
The clinical presentation in patients with multifocal musculoskeletal tuberculosis may closely mimic that in patients with multiple bone metastases, which makes the accurate clinical diagnosis challenging. Clinical and even radiological findings may be indistinguishable from malignant disease, so histopathological evaluation is warranted in such cases. Read More

Authors:  Regmi Pradeep,  Amatya Isha

Keywords:  Multifocal osteomyelitis ,  Tubercular ,  Children

Wei Xin,  Thapa Mahesh,  Reed Robyn

Final Pr. ID: Poster #: CR-020

A 14 year old male presented with a palpable, growing bump on the dorsal aspect of his left thumb since 1 year prior. Radiographs showed a large soft tissue mass dorsal to the interphalangeal joint of the left thumb with extension into the proximal phalanx. Contrast-enhanced MRI demonstrated the mass as mildly lobular and T2/STIR hyperintense, with internal septations and nodular wall enhancement. The lesion was biopsied and excised.

Final pathology results revealed an extraneural (soft tissue) perineurioma. Perineuriomas are very rare, usually benign peripheral nerve neoplasms composed of perineural cells. Because of the rarity of these lesions, advanced imaging with MRI becomes a useful tool in diagnosis, extent of disease, and surgical planning. Read More

Authors:  Wei Xin,  Thapa Mahesh,  Reed Robyn

Keywords:  perineurioma ,  perineuriomas ,  extraneural

Huang Jennifer,  Hernanz-schulman Marta,  Luo Yu

Final Pr. ID: Poster #: CR-021

Congenital syphilis (CS) results from transplacental or intrapartum infection by the spirochete bacterium Treponema pallidum. After a nadir in 2005, its incidence has been rising since 2013, currently affecting 48.5 infants per 100,000 live births; this represents a nearly 300% increase since 2015. However, CS remains infrequent, and may not receive appropriate consideration in the differential diagnosis. We report such a case in which the differential diagnosis was not initially entertained. Recognition of the imaging findings of this congenital infection by the pediatric radiologist can be critical in the timely management of this condition.

Case: A 4-month old boy had wrist swelling noted by his grandmother who took him to the local ED where he was found to have a right distal radial fracture. The infant was transferred to our tertiary referral pediatric institution with concern for non-accidental trauma. Skeletal survey at our institution demonstrated multiple variable-shaped and sized lytic lesions in bilateral femurs, tibias, right fibula, bilateral humeri, radii, ulnas, right scapula and multiple vertebrae. Suggested differential diagnosis included metastatic disease, LCH, leukemia or infantile myofibromatosis, which prompted oncological evalution. CS was added late to the differential diagnosis. Further questioning then revealed that patient's mother had had syphilis during pregnancy with unknown treatment history. Further physical examination revealed multiple skin lesions and mucosal ulcers, rash, and blisters. Patient's treponemal Ig G was positive and he was treated with penicillin with RPR(Rapid Plasma Reagin) titer follow-up.

CS has a high fetal or perinatal mortality rate approaching 40%. However, most live infected newborns are asymptomatic. The common clinical presentation includes fever, rash, rhinitis, hepatosplenomegaly, anemia, jaundice and elevated liver enzymes. The common skeletal findings include periosteal reaction, metaphyseal lucent bands, widespread metaphyseal erosions; particularly characteristic (although not specific) is the Wimberger sign, which irefers to the bilateral destruction of the proximal medial tibial metaphyses with sparing of a few medial millimeters (Laval-Jeantet collar). Differential diagnosis of CS includes osteomyelitis, neuroblastoma/metastases, leukemia, infantile fibromatosis, NAT. Characteristic radiographic and clinical lesions will be presented with discussion of differential diagnoses. Read More

Authors:  Huang Jennifer,  Hernanz-schulman Marta,  Luo Yu

Keywords:  syphilis ,  Congenital

Gould Sharon,  Thacker Mihir

Final Pr. ID: Poster #: CR-022

Knee deformity is the most common and complex lower extremity abnormality associated with Thrombocytopenia Absent Radius (TAR) syndrome. Conventional pre-operative imaging includes radiographs and computed tomography (CT) for assessment of joint alignment. We report utilizing 3-D MRI series and manual segmentation on commeicially available software to create 3-D printed models for pre-operative planning in a TAR syndrome patient with largely unossified epiphyses who had unusually severe femoral anteversion and genu varum. We discuss the methods used for imaging and segmentation as well as the value and limitations of the 3D print in pre-operative planning for this case. Even with the limitations we encountered, better understanding of the spatial relationships and joint alignment was achieved with 3-D model generation and aided in planning for correction of the knee varus deformity and femoral torsion. In addition, the diagnostic MRI information provided the basis to forgo construction of cruciate ligaments at this stage due to an increased risk of failure related to severe joint deformity. Because the prognosis for TAR syndrome is good if the child survives the first 2 years, it is important that orthopedic interventions are well planned to give a good outcome. Utilization of advanced imaging tools such as 3D imaging and printing may aid in definitive surgical planning in complex cases such as this one, and MRI can be used to generate usable anatomical models for pre-operative planning in children with incompletely ossified epiphyses. Read More

Authors:  Gould Sharon,  Thacker Mihir

Keywords:  3D printing ,  MRI ,  segmentation

Ratnayake Charith,  Subramanian Subramanian,  Narayanan Srikala,  Gaesser Jenna

Final Pr. ID: Poster #: CR-023

We present novel brain MRI findings in 2 children presenting with seizures due to TMCO1 mutation. Both children had absent olfactory bulbs and olfactory tracts and hippocampal malrotation. EEG demonstrated right temporal slow waves and intermittent focal slowing. One child had pontine hypoplasia, hypoplasia of bilateral optic nerves/chiasm, and an absent right cochlear nerve. In both children, there was increased mineralization in bilateral globi pallidi and bilateral substantia nigra on SWI sequence, possibly from excessive calcium. Ventriculomegaly and cavum septum pellucidum were also present in both children. Spine MRI revealed segmentation abnormalities including partial fusion of T2/T3 vertebral bodies and fusion of the posterior elements of T3-T5. Both children exhibited dysplastic and bifid ribs, demonstrating partial posterior fusion. Right renal agenesis was also noted in one child.

TMCO1 mutation results in cerebro-facio-thoracic dysplasia (CFTD) due to abnormal calcium homeostasis. The transmembrane and coiled-coil domains 1 protein directs formation of endoplasmic reticulum calcium leak channels which facilitate calcium leak upon overload of the endoplasmic reticulum. Failure of calcium leak results in abnormal cell function resulting in delayed osteogenesis, reduced mitochondrial volume, reduced mitochondrial respiration, and decreased endoplasmic reticulum stress mediated apoptosis. These children have distinctive craniofacial dysmorphism, global developmental delay, and skeletal anomalies. Previous studies have demonstrated only mild ventriculomegaly, corpus callosum abnormalities, frontotemporal atrophy, and three cases of associated epilepsy. Olfactory bulb agenesis may be due to abnormal development of the cribriform plate of the ethmoid and failure of olfactory nerves to induce development of the olfactory bulb from the telencephalon. We demonstrate that TMCO1 may play a more extensive and previously undescribed role in neurodevelopment, specifically in the formation of the hippocampus, optic nerve, and pons. Read More

Authors:  Ratnayake Charith,  Subramanian Subramanian,  Narayanan Srikala,  Gaesser Jenna

Keywords:  Pediatric ,  Dysplasia ,  MRI

Vorona Gregory,  Mahdi Eman,  Ritter Ann,  Henry Chrisopher,  Rao Sanjai,  Richard Hope,  Urbine Jacqueline

Final Pr. ID: Poster #: CR-024

The purpose of our case series is to review the imaging, clinical, and pathologic findings of two adolescent patients that presented to our medical center who were ultimately found to have the juvenile/young adult (Prust Type II) form of Alexander Disease.

The first patient was an eleven year old male with presumed conversion disorder who was transferred from a pediatric residental mental health clinic after a suspected aspiration event. The parents reported that the patient had difficulty swallowing for years, that had worsening significantly over the preceding six months. A non-enhanced head CT was performed shortly after the patient was admitted due to a change in mental status, which revealed a focal abnormality at the cervicomedullary junction. Subsequent assessment with MRI confirmed the lesion, which demonstrated corresponding T2/FLAIR signal hyperintensity and enhancement, with the presumptive initial diagnosis of a cervicomedullary tumor. The patient was biopsied at an outside institution, and was diagnosed with Alexander Disease by that institution.

The second patient was a twelve year old male with history of cleidocranial dysplasia, in which extensive signal abnormality and enhancement was first identified throughout the posterior fossa structures on an outpatient MRI of brain obtained for paroxysmal episodes of dizziness. A small amount of signal abnormality in the periventricular white matter was also present. The patient was initially worked up and treated for a neuroinflammatory disorder, and a biopsy was performed when there was further worsening of the patient’s symptoms. A mutation in the patient's GFAP gene was subsequently identified of “uncertain significance”.

Juvenile/young adult (Prust Type II) Alexander Disease is a rare leukodystrophy, which is associated with a different set of imaging characteristics compared with the more classic infantile/juvenile (Prust Type I) form. Radiologists who regularly interpret pediatric neuroimaging studies should be aware of its existence and its spectrum of associated imaging findings, in the interest of both arriving at the correct diagnosis and in avoiding unnecessary brain biopsy. Read More

Authors:  Vorona Gregory,  Mahdi Eman,  Ritter Ann,  Henry Chrisopher,  Rao Sanjai,  Richard Hope,  Urbine Jacqueline

Keywords:  Alexander ,  leukodystrophy

Haddad Sophie,  Foran Ann,  Tierradentro-garcia Luis,  Ho Fung Victor M,  Hwang Misun

Final Pr. ID: Poster #: CR-025

Hypoxic ischemic encephalopathy (HIE) is a significant cause of severe long term neurological impairment and mortality in the newborn. It develops in two stages; the ischemic phase caused by hypoxia leading to cytotoxic edema, followed by the reperfusion phase with the release of reactive oxygen species leading to oxidative damage and vasogenic edema. Imaging plays a key role in diagnosis, management, and treatment of HIE. It also offers important prognostic information. MRI remains the preferred imaging modality.
Microvascular imaging (MVI) is a novel advanced Doppler ultrasound (US) technique that permits visualization of the microvasculature without the administration of contrast agents. It enhances the visualization of flow signal in the microvessels and helps reveal functional insights into the brain at high resolution.

A female newborn of 32 weeks of gestation was delivered by urgent C-section following absent fetal movements and suspected placental abruption. She was born to a mother with a reported exposure to cocaine with a last use the week prior to delivery. At birth, no pulse was detected, and CPR was initiated immediately. O2 saturation remained low during resuscitation despite intubation and a FiO2 of 100%. Return of spontaneous circulation was noted 50 minutes after birth. Following birth, the patient was diagnosed with severe HIE and had multiple seizures. Her physical exam revealed fixed and cloudy pupils with a diameter of 4 mm and no brainstem reflexes. At one day of life, a brain US was done and displayed diffusely increased echogenicity of the parenchyma and crowding of the cortical gyri concerning for cerebral edema. MVI revealed markedly increased microvascular perfusion in the basal ganglia, thalami, and periventricular region consistent with post-ischemic reperfusion in the setting of HIE. MRI showed diffusion restriction throughout the brain including the medial temporal lobes, hippocampi, thalami, basal ganglia, and frontoparietal cortex. Also noted was extensive hyperintense signal abnormality on T2-weighted sequences within both the gray and white matter with loss of the gray-white matter differentiation and moderate sulcal effacement. These findings confirmed the diagnosis of severe HIE.

In our case, MVI demonstrated marked hyperperfusion in the basal ganglia, thalami and periventricular region. Whether this MVI flow signature can prognosticate poor clinical outcomes, it warrants further investigation. Read More

Authors:  Haddad Sophie,  Foran Ann,  Tierradentro-garcia Luis,  Ho Fung Victor M,  Hwang Misun

Keywords:  Microvascular Imaging ,  Hypoxic ischemic encephalopathy

Venkatakrishna Shyam Sunder,  Andronikou Savvas

Final Pr. ID: Poster #: CR-026

Chiari Type I deformity (C1) is associated with bony deformity of the skull base and herniation of cerebellar tonsils. MRI is used for diagnosis and surgery is advised for symptomatic children. We present a case series using MR imaging including CSF flow, in a variety of children with C1 to demonstrate a variety of outcomes both surgical and non-surgical: spontaneous resolution, spontaneous worsening, post-surgical improvement.

Case 1: A 6-year-old (y) girl referred for imaging with short stature and growth hormone deficiency demonstrated incidental findings of C1 without syringomyelia. No surgery (Sx) was performed, and follow-up (F/U) MRI at age 7 y demonstrated spontaneous resolution of the tonsillar ectopia and expansion of the CSF spaces at craniocervical junction (CCJ).

Case 2: A 7 y boy with headaches and staring spells underwent an MRI demonstrating 6 mm protrusion of pointed cerebellar tonsils and CSF space reduction at CCJ. No Sx was performed, and F/U imaging at age 9 y demonstrated spontaneous improvement in cerebellar tonsillar position and increased bidirectional CSF flow at CCJ.

Case 3: A 2-week-old girl underwent brain MRI demonstrating an ectopic neurohypophysis, under opercularization suggesting brain immaturity and a normal CCJ. F/U MRI at 6 y of age showed interval spontaneous development of C1 with decreased CSF spaces at CCJ.

Case 4: A 17-month-old boy underwent brain MRI for unsteady gait and poor vestibular response, which showed C1 and narrow CSF spaces at the foramen magnum and with reduced CSF flow. At age 3 y, after posterior fossa decompression, F/U MRI showed post-surgical improvement of the position of the cerebellar tonsils and increased CSF space at CCJ.

Case 5: A 8 y girl with headaches and intermittent dizziness underwent MRI demonstrating 15 mm protrusion of the pointed cerebellar tonsils and marked decrease in the CSF at CCJ. After suboccipital craniotomy, MRI at age 8 y demonstrated a post-surgically improved CSF space and improved bidirectional CSF flow, at CCJ. Read More

Authors:  Venkatakrishna Shyam Sunder,  Andronikou Savvas

Keywords:  Chiari Type I deformity (C1) ,  CSF space ,  CSF flow

Gaddam Nikhita

Final Pr. ID: Poster #: CR-027

Tubercular infection of spine occurs in the form of tuberculous spondylitis, intradural tuberculosis, and tubercular myelitis in the decreasing frequency. Intradural tuberculosis has been variously termed as intradural extramedullary tuberculosis, spinal arachnoididtis and chronic adhesive arachnoiditis. Spinal TB radiculomyelitis is a secondary TB lesion, although it may rarely occur primarily. This is a casereport of 18 year old male child who is a known case of TB meningitis and developed syringomyelia and arachnoiditis. Read More

Authors:  Gaddam Nikhita

Keywords:  arachnoiditis ,  syringomyelia

Kryger Marc,  Hernandez Alejandra,  Herring Katye,  Riegler Lara,  Hook Marcus,  Daugherty Reza

Final Pr. ID: Poster #: CR-028

A 16 year old boy reporting several months of hip pain, weight-loss, and fatigue presented to our institution after a pelvis radiograph ordered by his family physician showed concerning findings. MR imaging of his pelvis demonstrated a large heterogeneously enhancing mass arising from the right iliac wing causing significant mass effect on the pelvic organs and soft tissues of the buttocks. A percutaneous biopsy confirmed the diagnosis of Ewing sarcoma.
A staging CT of the chest was performed, demonstrating multiple lung metastases. In addition to this finding, there was multifocal bilateral nodular tumor thrombus propagating along multiple subsegmental pulmonary arteries, with a pseudo tree-in-bud morphology. Given their course, these metastatic deposits appeared endovascular in origin. Read More

Authors:  Kryger Marc,  Hernandez Alejandra,  Herring Katye,  Riegler Lara,  Hook Marcus,  Daugherty Reza

Keywords:  Ewing ,  Sarcoma ,  Metastasis

Hadian Fatemeh,  Jaker Sams,  Helm Emma

Final Pr. ID: Poster #: CR-029

Hyperimmunoglobulin E syndrome, or Job syndrome, is a rare primary immunodeficiency disorder characterised by a clinical triad of eczema, recurrent skin and lung infections, and high serum IgE that often presents in childhood. Hyper IgE syndrome is most commonly caused by a STAT3 genetic mutation, which impairs the appropriate inflammatory response leading to recurrent infections. Respiratory infections are usually staphylococcal and less commonly due to H. Influenzae and S. pneumoniae. Complications include lung abscess formation, bronchiectasis, pneumatocoeles and secondary opportunistic infections including aspergillosis.

We present radiographic and CT imaging findings of a series of three pediatric patients who presented to our hospital with varying degrees of severity and complications.

Case 1 is a 3 year old child who initially presented via dermatology with a postauricular abscess and recurrent scalp infections. They went on to develop a large multiloculated pneumatocoele further complicated by a large hydropneumothorax.

Case 2 is a 4 year old child who presented with eczema since infancy; further history revealed multiple staphylococcal abscesses including a liver abscess at 6 months of age requiring ITU admission. Imaging revealed cystic bronchiectasis and multiple pneumatocoeles. They went on to be investigated for fungal lung infection.

Case 3 is an 11 year old child who presented with respiratory tract symptoms and pleuritic chest pain. Imaging revealed large lung abscesses, which resolved over serial imaging. They went on to develop varicose bronchiectasis and allergic bronchopulmonary aspergillosis.

Early diagnosis is crucial in children to preserve respiratory function and improve longterm survival. The radiologist should be aware of the radiological manifestations and complications of Hyper IgE syndrome, particularly in the respiratory tract, as complications of respiratory infection are among the most common causes of mortality. Read More

Authors:  Hadian Fatemeh,  Jaker Sams,  Helm Emma

Keywords:  Job ,  hyperimmunoglobulin

Gagnon Marie-helene,  Richer Edward,  Alazraki Adina

Final Pr. ID: Poster #: CR-030

A 16 year old previously healthymale patient who initially presented with daily recurrent fevers and chills lasting 5-10 minutes. His parents brought him to a tertiary care hospital after developing vomiting and weight loss in the setting of progressively worsening fevers. Initial work up was remarkable for elevated CRP and a negative infectious workup. An MR enterography ordered to evaluate for a cause of weight loss demonstrated incidental lung base lesions. Follow up CTA revealed a lesion within the right ventricle and multiple lung nodules, concerning for endocarditis and septic emboli. He underwent two surgical resections over two months for the ventricular mass. Pathology revealed findings consistent with necrotizing endocarditis. During his hospital course, he was found to have a left cephalic venous thrombosis and was placed on Xarelto, later developing a right subclavian vein thrombus while on anticoagulation. He was readmitted 1 month later after presenting for right chest and right upper quadrant pain. Initial chest X-ray revealed right lower lobe mass and follow up CTA revealed two large right pulmonary artery pseudo-aneurysms. He underwent cardiac catheterization for pre-operative coiling of the pseudo-aneurysms and subsequent right lower lobectomy. Pathology revealed necrotizing arteritis and pulmonary artery pseudo-aneurysms. Based on clinical presentation and further work up, he was diagnosed with Hughes Stovin Syndrome and started on Cytoxan and Apixaban. While not yet considered in remission, his symptoms have improved since initiating treatment.

Hughes Stovin Syndrome (HSS) is a rare disorder characterized by thrombophlebitis as well as multiple pulmonary and/or bronchial aneurysms. There is a male predilection, usually presenting between 12-48 years. There have been less than 40 cases published in the English literature. HSS is thought to be a variant of Bechet’s but does not present with oral or genital ulcers. The lack oral and genital ulcers in this patient excluded a diagnosis of Bechet’s. The underlying cause of HSS is unclear but is believed to be due to angiodysplasia or infection. Since there is no formally described diagnostic criteria, the clinical presentation of thrombophlebitis with pulmonary artery aneurysms characterize the disease.

We present this case to illustrate the clinical presentation of a rare and possibly under recognized syndrome. Read More

Authors:  Gagnon Marie-helene,  Richer Edward,  Alazraki Adina

Keywords:  Vasculitis ,  Aneurysm ,  Thrombophlebitis

Suryaningrat Windu,  Amal Mohamad,  Sekarsari Damayanti

Final Pr. ID: Poster #: CR-031

The bridging bronchus is a rare bronchus anomaly which mostly found in infants and children. Aberrant bronchus that arises from either right or left main bronchus and crosses the mediastinum to supply the collateral lung lobe has come to the term of bridging bronchus. The diagnosis is often challenging as the bridging bronchus itself can be asymptomatic or presented by recurrent respiratory distress, but the use of right protocol in cross-sectional imaging is helpful enough to find the bridging bronchus.
This case report aims to present a case of a 5-month-old boy with recurrent pneumonia. He experienced episodes of severe dyspnea, fever, and desaturation, with negative PCR for covid-19. His echocardiography showed a moderate ventricular septal defect (VSD), worsening his dyspnea episodes which were accompanied by the decrease of oxygen saturation to 92% in room air. He underwent VSD closure procedure, which omitted the desaturation episodes, yet still experienced recurrent episodes of dyspnea and fever within 4 months of follow-up. In each episode, he had to be hospitalized and received conventional therapy by using antibiotics, bronchodilators, corticosteroids. Computed tomography (CT) scan of thorax using the pediatric radiation dose was done to evaluate the cause of recurrent pneumonia. Imaging findings in post-contrast slices (taken in inspiration phase) showed no connection between right lower lobe and left main bronchus, which was then suspected as esophageal bronchus. Then, in pre-contrast slices (taken in expiration phase) showed branch of the trachea into a right main bronchus and a stenotic aberrant bronchus (bridging bronchus, type 2) to the left hemithorax, and branching as right lower lobe bronchus and left main bronchus. Further evaluation of all slices in CT scan of thorax showed the stenotic bridging bronchus occluded in post-contrast slices, which were accidentally taken in inspiration phase. The occluded bridging bronchus led to non-optimal ventilation of the left lung lobes and right lower lobe, causing an imbalance of mucus clearance. Thus, increases the risk of mucus accumulation and rise the bacterial risk of infection which manifests as recurrent pneumonia in this patient. Nevertheless, within four months of follow up the patient was discharged with stable condition and symptoms-free. Read More

Authors:  Suryaningrat Windu,  Amal Mohamad,  Sekarsari Damayanti

Keywords:  bridging bronchus ,  congenital airway anomaly ,  airway anomaly

Sodhi Kushaljit,  Bhatia Anmol,  Gauba Richa,  Mathew Joseph L,  Bal Amanjit

Final Pr. ID: Poster #: CR-032

A 9-year-old girl presented with lethargy, fatigue and progressive exertional dyspnea for the last two-three years, and rapid breathing for the past six months. Chest X-ray showed confluent air space opacities in both lungs. Chest computed tomography (CT) scan done outside 3 months previously showed diffuse ground-glass opacities with interstitial thickening suggestive of crazy paving in bilateral lungs. To evaluate the disease progression without the radiation exposure associated with another CT scan, magnetic resonance imaging (MRI) was performed. It showed diffuse hyperintense areas bilaterally on T2 turbo spin echo and MultiVane XD sequences, indicative of ground-glass changes. Radiologically, a possibility of pulmonary alveolar proteinosis (PAP) was given. Subsequently, bronchoalveolar lavage and lung biopsy confirmed PAP.

In PAP, the phospholipidoproteinaceous material which deposits within the alveoli presents with longer T2 relaxation time, and longer or equal T1 relaxation time which results in hyperintense signal, as seen in the index case. With the recent advances in lung MRI, short-duration protocols are able to give diagnostic information similar to CT in children [1-2], thereby emerging as an attractive radiation-free modality. MRI can demonstrate the findings of PAP similar to CT, and may be relatively better than CT in picking up the parenchymal changes associated with PAP [3].

To conclude, MRI may serve as an attractive radiation-free modality in diagnosing PAP in children and evaluating the lung changes over time.

REFERENCES:
1. Sodhi KS, Khandelwal N, Saxena AK, et al. Rapid lung MRI in children with pulmonary infections: Time to change our diagnostic algorithms. J Magn Reson Imaging. 2016;43:1196-206.
2. Sodhi KS, Khandelwal N, Saxena AK, et al. Rapid lung MRI - paradigm shift in evaluation of febrile neutropenia in children with leukemia: a pilot study. Leuk Lymphoma. 2016;57:70-5.
3. Luo J, Yang D, Xiao E, et al. Lung MRI at 3T: comparison of CT and MRI in initial evaluation of pulmonary alveolar proteinosis. Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2013;38:1160-6. Read More

Authors:  Sodhi Kushaljit,  Bhatia Anmol,  Gauba Richa,  Mathew Joseph L,  Bal Amanjit

Keywords:  PAP ,  MRI ,  CT

Stern Joseph,  Ramirez Suarez Karen,  Victoria Teresa,  Otero Hansel

Final Pr. ID: Poster #: CR-033

Hepatopulmonary fusion (HPF) is a very rare type of right-sided congenital diaphragmatic hernia (CDH) with a reported prevalence of 3 in 1000 right-sided CDH cases. By June 2020, less than 50 cases have been reported in the literature. Out of 147 patients with right CDH managed at our institution, we found only one case (0.68%) of HPF, which is presented here.

HPF is typically not identified preoperatively, and the presence of HPF changes the operative management. It has been reported that a large right CDH without leftward cardiomediastinal shift and atelectatic lung adherent to the liver are imaging features of HPF.

This patient was a full-term, newborn girl with a normal prenatal course who developed respiratory distress immediately after birth. Chest radiograph at birth demonstrated a right CDH. On day four of life, the patient underwent surgical repair of right CDH. During the procedure, hepatopulmonary fusion involving the cephalad portion of the liver was discovered. The lung and liver could not be separated safely so the diaphragmatic defect was closed around the portion of the liver that extended into the thorax. Postoperative CT angiography of the chest and abdomen demonstrated no abnormal vascular communication between the lungs and liver; however, anomalies of systemic veins were identified. Follow-up chest radiograph at 9 months of age demonstrated a large right CDH that did not include bowel or right-sided colon and was without leftward mediastinal deviation. Concurrent contrast-enhanced CT images of the thorax at this time confirm the right CDH with herniated liver occupying most of the right hemothorax and expected rightward traction of the liver vasculature. However, no significant leftward mediastinal deviation was noted. A small amount of atelectatic lung overlying the liver was also noted, another imaging sign expected with HPF. Last follow-up at age 4, patient was thriving with normal growth and development. Read More

Authors:  Stern Joseph,  Ramirez Suarez Karen,  Victoria Teresa,  Otero Hansel

Keywords:  Hepatopulmonary Fusion ,  Congenital diaphragmatic hernia ,  CTA