López Diana,  Arrate Antonia,  Leal Camilo,  Méndez Daniela,  Pérez-marrero Lizbet

Final Pr. ID: Poster #: CR-043

Case report: Congenital bronchobiliary fistula is a rare congenital malformation, with high morbidity and mortality. It is characterized by a communication between the respiratory and digestive tracts, between a bronchus and the bile duct.
This is a full-term female newborn patient, appropriate for gestational age, who from the third day of life presents with progressive respiratory difficulty associated with abundant waste through a nasogastric tube. She evolved with greater respiratory difficulty, so she was kept on a complete bowel rest and with a nasogastric tube, which increased his output. Airway aspiration was suspected, and an esophagus-stomach-duodenum study was performed to evaluate gastroesophageal reflux, which was normal. The study continued with abdominal ultrasound, showing aerobilia.
The patient evolves with severe respiratory distress and requires mechanical ventilation on day 15, so a chest tomography is performed, which shows aerobilia and a communicating tract between the right main bronchus and the bile duct.
Surgical management was performed with closure of the proximal end of the fistula at the supradiaphragmatic level, without intervening the intrahepatic tract.
The newborn later develops cholestatic syndrome, which is studied with cholangiopancreatography magnetic resonance (MRCP) that reports atresia of the extrahepatic bile ducts, performing an entero-biliary anastomosis in a second surgical procedure.
During her childhood after being discharged, she was diagnosed with several obstructive bronchial syndromes, cholangitis and malnutrition, remaining stable at the outpatient level. She is currently 15 years of age, her only chronic illness being with chronic liver disease, without evidence of clinical or elastographic fibrosis.
Bronchobiliary fistula presents as bronchitis and repeated bronchopneumonia, bilioptysis, cholangitis and sepsis in some cases. Proposed etiologies are either union of an accessory bronchus with an aberrant bile duct or duplication of the foregut. This diagnosis can be associated with other malformations such as bile duct atresia and right diaphragmatic hernia. Computed tomography with 2D and 3D reconstructions and MRCP are recommended for diagnostic workup. Read More

Authors:  López Diana , Arrate Antonia , Leal Camilo , Méndez Daniela , Pérez-marrero Lizbet

Keywords:  Bronchobiliary fistula, Congenital malformation, Bilioptysis

Murphy Ryan,  Murati Michael,  Holm Tara,  Dietz Kelly

Final Pr. ID: Poster #: EDU-012

The presacral space is composed of multiple tissue types, including osteochondral, mesenchymal, neurogenic, vascular and lymphatic. The presence of these tissues leads to a long and complex differential for a presacral mass in a pediatric patient. Specifically, the differential also includes anterior sacral meningocele, enteric cyst, vascular malformations, neuroblastoma, ganglioneuroma, schwannoma, neurofibroma, rhabdomyosarcoma, lymphomatous masses, giant cell tumor, aneurysmal bone cyst, osteosarcoma, Ewing sarcoma, and chordoma. Imaging plays a key role in characterizing these masses and treatment planning. Familiarity with the common presacral masses of infancy and childhood is therefore necessary for the pediatric and general radiologist.

Once an osteochondral or neurogenic mass is excluded, and a predominantly cystic presacral mass is present, the primary differential consists of a collection of developmental or congenital masses including sacrococcygeal teratoma, anterior meningocele, low flow vascular malformation, and an enteric duplication cyst. We will review the common imaging features and associations of these cystic masses by presenting a series of cases. Additional examples of solid and osteochondral presacral masses will be included where appropriate for comparison in order to avoid characterization pitfalls and highlight teaching points. Read More

Authors:  Murphy Ryan , Murati Michael , Holm Tara , Dietz Kelly

Keywords:  anterior meningocele, low flow vascular malformation, enteric cyst

Bellew Elizabeth,  Sonstegard Anna Marie,  Finelt Nika,  Goldfisher Rachelle

Final Pr. ID: Poster #: CR-004

A full-term male was born with a 7 x 7 cm subcutaneous mass with central dark blue discoloration and peripheral erythema on the right flank/buttock. Initial hip and pelvis radiograph at one day old did not show evidence of calcifications. An ultrasound demonstrated an ill-defined, heterogeneous mass with internal vascularity with invasion of underlying muscle. MRI of the abdomen and pelvis with contrast at two days of age showed a T2 hyperintense mass with mild postcontrast enhancement within the right flank with infiltration of the underlying muscles including the right psoas, iliacus, gluteal, and the left erector spinae. The differential diagnosis included: kaposiform hemangioendothelioma, rhabdomyosarcoma (RMS), tufted angioma and neuroblastoma.

Given the broad list of differential diagnoses, a core biopsy of the right buttocks was obtained at 6 days of age to further characterize the lesion. Hematoxylin and eosin stained sections demonstrated highly collagenized and sclerotic spindle cells involving the adipose tissues extending into the dermis. Immunohistochemical stained sections showed positivity to desmin, myogenin and MyoD1. Fluorescence in situ hybridization studies indicated the presence of a VGLL2 gene rearrangement. These pathologic findings were consistent with sclerosing spindle cell RMS. At 13 days old, a PET scan showed the right flank lesion with minimal to no FDG uptake, a finding which is consistent with sclerosing RMS given that this sub-type consists mainly of collagen. There were no pulmonary nodules. After multiple chemotherapy cycles, subsequent MRIs of the abdomen and pelvis showed no residual enhancing lesion. The patient will be closely followed for local recurrence.

RMS, the most common soft tissue tumor seen in children, are mesenchymal tumors of skeletal muscle and are most often seen in head and neck, but are less commonly noted on the flank. Sclerosing spindle cell RMS is a subtype often seen in childhood, but exceedingly rare in newborns. Cutaneous RMS should be considered in the differential diagnosis of a large subcutaneous lesion in a newborn. Read More

Authors:  Bellew Elizabeth , Sonstegard Anna Marie , Finelt Nika , Goldfisher Rachelle

Keywords:  Rhabdomyosarcoma, Sclerosing Spindle Cell Rhabdomyosarcoma, Vascular Malformation

Gual Fabiana,  Lima Natalia,  Matsuoka Marcia,  Sameshima Yoshino

Final Pr. ID: Poster #: EDU-065

Vascular anomalies represent a spectrum of disorders ranging from simple "birthmarks" to life-threatening entities. Incorrect nomenclature and misdiagnosis are common in patients with these anomalies. The objectives of this educational presentation are to provide radiologists with a comprehensive understanding of the ISSVA (International Society for the Study of Vascular Anomalies) classification and to enhance their familiarity with the clinical and imaging features of commonly encountered soft tissue vascular anomalies in pediatric patients. Special emphasis is placed on emphasizing the practicality of ultrasound as an effective diagnostic tool. The ISSVA has been updated with the recognition of causal genetic mutations, most recently revised in May 2018. The main organizational principle of this classification divides vascular lesions into: vascular tumors (neoplastic): benign, locally aggressive/borderline, and malignant; vascular malformations (non-neoplastic): simple, combined, major named vessels, and associated with other anomalies; unclassified anomalies (unclear whether tumor or malformation). Read More

Authors:  Gual Fabiana , Lima Natalia , Matsuoka Marcia , Sameshima Yoshino

Keywords:  vascular anomalies, hemangioma, vascular malformations

Gaballah Marian,  Goldfisher Rachelle

Final Pr. ID: Poster #: EDU-096

Lymphatic malformations (LMs) are low-flow vascular malformations which are composed of dilated lymphatic channels, forming septated cyst-like structures (2). LMs are the second most common type of vascular malformation, second to venous malformations (1). The most common locations are in the neck, followed by the axillary region. On MRI, LMs are multiloculated, T2 hyperintense lesions, which may have fluid-fluid levels, and are without flow voids (1, 2). They can involve multiple tissue planes and do not regard anatomical and fascial boundaries (2). Cystic lymphatic malformations are further divided into microcystic, macrocystic, or mixed, based on the size of their cystic components. Macrocystic LMs are composed of larger cysts, while microcystic LMs are composed of smaller cysts and may appear solid on imaging.

We present ten cases of microcystic, macrocystic, and mixed lymphatic malformations in a variety of anatomical locations. In addition to demonstrating the imaging findings, we present a review of the literature in regards to each anatomical region. Lymphatic malformations in this presentation include right orbit (n=1), mediastinum (n=2), pulmonary bronchovascular bundles/pleural space (n=1), retroperitoneum (n=1), mesentery (n=2), perirectal and scrotal (n=1), lower extremity (n=2). 6 of these children also had additional sites of T2 hyperintense disease involving the bones or spleen, suggestive of additional lymphangiomas.

This presentation summarizes ten cases of lymphatic malformations in a variety of less common anatomical locations and a review of the pertinent literature.

References:

1. Flors L, Leiva-Salinas C, Maged IM et al. (2011) MR Imaging of Soft-Tissue Vascular Malformations: Diagnosis, Classification, and Therapy Follow-up. Radiographics 31:1321-1340.
2. White CL, Olivieri B, Restrepo R et al. (2016) Low-flow vascular malformation pitfalls: from clinical examination to practical imaging evaluation- part 1, lymphatic malformation mimickers. AJR 206: 940 – 951. Read More

Authors:  Gaballah Marian , Goldfisher Rachelle

Keywords:  Lymphatic malformation, Vascular Malformation

Kinariwala Dhara,  Daugherty Reza,  Park Auh Whan

Final Pr. ID: Poster #: EDU-009 (S)

Comprehensive radiologic evaluation and accurate diagnosis of vascular malformations is vital to guide optimal treatment. Technical approaches and sclerotic agents used by interventional radiology vary by the type of malformation, anatomic location, and architecture of the malformation. Proper pre-procedural characterization of hard-to-treat lesions impacts procedural success and long-term outcomes.

Most vascular malformations located superficially on the extremities and trunk, and can be evaluated with ultrasound and color Doppler. However, evaluation can be complicated by unusual locations and difficulty in characterization, requiring MRI and/or MRA. We present interesting pediatric vascular malformations challenging for both imaging diagnosis and treatment.

The objectives of this poster include:
1. To review the classification and nomenclature of vascular malformations.
2. To present multimodal imaging findings of vascular malformations.
3. To discuss diagnostic imaging approaches of vascular malformations.modalities used.
4. To describe the treatment approaches for these malformations and outcomes including technical considerations and follow up. Read More

Authors:  Kinariwala Dhara , Daugherty Reza , Park Auh Whan

Keywords:  Vascular Malformations

Bhalla Deeksha,  Jana Manisha,  Manchanda Smita,  Bhalla Ashu,  Naranje Priyanka

Final Pr. ID: Poster #: EDU-069

Teaching points:

The spectrum of neck masses in neonates and infants (< 2 years) differs considerably from those in older children
Understand characteristic imaging appearances, particularly recognise entities that do not require sampling for diagnosis
Learn algorithmic approach to differential diagnosis based on age and lesion morphology with case based examples

Table of contents:
Introduction: Incidence, clinical considerations
Classification:
Age: Neonate
Cystic
Branchial cleft cyst
Dermoid cyst
Thyroglossal duct cyst
Solid:
Germ cell tumor
Congenital hemangioma
Neuroblastoma
Mixed
Primitive myxoid mesenchymal tumor (PMMT)
Teratoma

Age: Older infants
Cystic
Branchial cleft cyst
Dermoid cyst
Thyroglossal duct cyst

Solid:
Lymphoma
Granulocytic sarcoma
Rhabdomyosarcoma
Multinodular vacuolating tumor of infancy (MNTI)
Solitary fibrous tumor (SFT)
Fibrous tumors: Fibrous hamartoma of infancy, infantile fibrosarcoma

Vascular malformation (microcystic lymphatic, venolymphatic, arteriovenous)

Infections
Ludwig angina
Zygomycosis

Practical diagnostic algorithms based on age, location (involved neck space) and morphology
Conclusion Read More

Authors:  Bhalla Deeksha , Jana Manisha , Manchanda Smita , Bhalla Ashu , Naranje Priyanka

Keywords:  Neck tumor, congenital, vascular malformation

Kinariwala Dhara,  Daugherty Reza

Final Pr. ID: Poster #: EDU-078

Although rare, CLOVES syndrome, a PIK3CA-related congenital overgrowth disorder, presents radiologists with opportunity for vital diagnostic and treatment planning. CLOVES syndrome is characterized by Congenital Lipomatous Overgrowth of the trunk, Vascular malformations, Epidermal naevi, and Skeletal and Spinal anomalies. Multimodal imaging findings can help distinguish CLOVES from other overgrowth syndromes such as Proteus syndrome and Klippel-Trenauny syndrome. We present multimodal imaging of four patients with CLOVES syndrome which demonstrate characteristic findings:

- Thoracic lipomatous hyperplasia, a key sign of CLOVES syndrome in which predominantly thoracic lipomatous masses grow in the subcutaneous tissues and invade the pleura, mediastinum, and upper abdomen, often with superficial vascular malformations
- Renal anomalies, including agenesis, hypoplasia, hydronephrosis, and cysts.
- Vascular malformations, including venous, venolymphatic, and lymphatic malformations
- Spinal cord defects, including spina bifida, medullary arteriovenous shunts, and congestive myopathy of the paravertebral venous plexus

Management of CLOVES syndrome focuses on debulking of lipomatous masses, treatment of clinically significant vascular malformations, and medical management of renal and neurologic sequelae. To that end, the role of the radiologist is accurate diagnosis of the syndromic pattern, isolation of the extent of lipomatous masses for pre-operative planning, and identification of renal and spinal cord anomalies.

The purpose of the poster is to:

1. Briefly review the types of congenital overgrowth disorders as demonstrated in various imaging modalities.
2. Focus on characteristic imaging findings of CLOVES syndrome.
3. Review the benefits and disadvantages of various imaging modalities.
4. Identify the most relevant radiologic findings for surgical, interventional and medical management.


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Authors:  Kinariwala Dhara , Daugherty Reza

Keywords:  Congenital, Thoracic, Vascular Malformations

Ugas Charcape Carlos,  Cerron Vela Carmen

Final Pr. ID: Paper #: 016

To characterize the range of cardiac and extracardiac CT findings in children with thoracic ectopia cordis in the setting of Cantrell syndrome.
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Authors:  Ugas Charcape Carlos , Cerron Vela Carmen

Keywords:  Pentalogy Of Cantrell, Cardiac, Malformation

Close Orrie,  Squires Judy

Final Pr. ID: Poster #: EDU-097

There are numerous congenital anomalies of the gastrointestinal tract with varying frequency. Some anomalies are life-threatening and require prompt diagnosis with immediate treatment. Evaluation of patients with these anomalies can be complex, sometimes requiring multiple imaging modalities for accurate diagnoses and timely treatment. It is therefore essential for radiologists to have a firm understanding of specific imaging features. The purpose of this exhibit is to review common and uncommon congenital anomalies of the gastrointestinal tract, including clinical and imaging features as well as treatment options. Read More

Authors:  Close Orrie , Squires Judy

Keywords:  congenital, gastrointestinal, malformations

Funaro Kimberly,  Kucera Jennifer

Final Pr. ID: Poster #: EDU-033

Congenital chest lesions encompass a wide array of pathology with overlapping imaging characteristics. With prenatal ultrasound and MRI, more congenital chest lesions are being detected sooner, and it is important for radiologists to be familiar with their imaging findings for both diagnostic and prognostic purposes. This educational exhibit reviews the spectrum of pediatric congenital chest lesions and their key imaging findings.
Read More

Authors:  Funaro Kimberly , Kucera Jennifer

Keywords:  Chest, Congenital malformations

Plut Domen,  Zupancic Ziva,  Mali Brajovic Senja,  Kljucevsek Tomaz,  Tomazic Mojca,  Glusic Mojca,  Homan Matjaz

Final Pr. ID: Poster #: CR-006

Congenital portosystemic venous shunts (CPVS) are rare vascular abnormalities in which the portal blood drains into a systemic vein. They are results of embryogenetic alterations during the complex development of the portal system in early gestational period. Anatomically they are classified into two groups; extrahepatic and intrahepatic. Extrahepatic shunts were first described in 1793 by Abernethy and are thus also called Abernethy malformations. Presentation of CPVS can be highly variable and consequences severe.

CASE1:
An 8 year old boy was admitted to our hospital due to severe abdominal pain. Doppler ultrasound (US) revealed an abnormal intrahepatic tubular vascular structure. The computed tomography angiography (CTA) confirmed a direct vascular connection between the left portal vein and the dilated left hepatic vein. The right portal vein was small in size, indicating main portal blood flow through the left side bypassing the liver. The treatment for this intrahepatic CPVS was embolization of the shunt. The boy has been well since the procedure.

CASE2:
A routine medical check-up in a 14 year old girl discovered abnormal liver function tests. Abdominal US showed a huge mass in the right lobe of the liver and a big mass of similar structure in the epigastrium. Portal vein was not identified. CTA showed a short main portal vein connected directly to the inferior vena cava, consistent with the Abernethy malformation. The big mass in the epigastrium was shown to be a tumor in the caudate lobe of the liver. Focal nodular hyperplasia (FNH) was considered for liver lesions due to the imaging findings and known association with the Abernethy malformation. Biopsy confirmed the diagnosis. The girl is currently waiting for liver transplantation, as it is the treatment of choice for this extrahepatic CPVS. Read More

Authors:  Plut Domen , Zupancic Ziva , Mali Brajovic Senja , Kljucevsek Tomaz , Tomazic Mojca , Glusic Mojca , Homan Matjaz

Keywords:  Congenital portosystemic venous shunt, Abernethy malformation, Intrahepatic shunt, Extrahepatic shunt

Hirsig Leslie,  Verma Nupur,  Sharma Priya,  Rajderkar Dhanashree

Final Pr. ID: Poster #: EDU-115

Congenital and childhood findings of pulmonary artery anomalies are uncommon but not infrequently encountered by referral centers. We present by case examples anomalies of pulmonary arterial vasculature, and discuss their clinical presentation, associated cardiothoracic anomalies, and overall prognosis. Read More

Authors:  Hirsig Leslie , Verma Nupur , Sharma Priya , Rajderkar Dhanashree

Keywords:  Pulmonary anamolies, Congenital malformations, Pulmonary Origin, Pulmonary Hypertension, Pulmonary complications

Wepking Kelly,  Gill Kara,  Riedesel Erica

Final Pr. ID: Poster #: CR-068

Currarino’s Syndrome (CS) is a unique form of caudal regression syndrome characterized by triad of sacral dysplasia, anorectal malformation, and presacral mass. Additional congenital malformations of the genitourinary tract and spine may be present.

Currarino's Syndrome has a variety of clinical presentations - from asymptomatic to severe chronic constipation - depending on associated malformations. Early identification and adequate treatment prevents eventual serious complications.

Plain radiograph of the sacrum is the first diagnostic step. Ultrasound or pelvic/spinal MRI is used to better evaluate for presacral mass. Fluoroscopic enema studies may be used for further evaluation of anorectal malformation. Additional imaging of the GU tract with US and VCUG is suggested due to risk for associated GU anomaly or vesicoureteral reflux.

We present two cases of Currarino’s Syndrome which demonstrate classic imaging findings on plain film radiograph, ultrasound, and MRI.

Case 1 presented in infancy with severe chronic constipation. Abdominal radiograph demonstrated classic "scimitar" sacrum. Fluoroscopic enema demonstrated high grade anal stenosis. US and MRI of the spine and sacrum revealed large anterior sacral meningocele.

Case 2 presented at birth with imperforate anus. Abdominal radiograph demonstrated classic "scimitar" sacrum. MRI of the spine and sacrum revealed small anterior sacral meningocele. Additional GU anomalies were discovered including vesicoureteral reflux and uterine didelphis. Read More

Authors:  Wepking Kelly , Gill Kara , Riedesel Erica

Keywords:  Currarino's Triad, Sacral dysplasia, Presacral mass, Anorectal malformation, Case Report

Emerson Miriam,  Barth Richard,  Rubesova Erika,  Halabi Safwan,  Rosenberg Jarrett,  Hintz Susan,  Blumenfeld Yair,  Girsen Anna,  Neves Stephanie,  Homeyer Margaret

Final Pr. ID: Poster #: SCI-039

Congenital Pulmonary Airway Malformation Volume Ratio (CVR) is widely used during prenatal ultrasound to predict outcome of fetuses with bronchopulmonary malformations (BPM). Since fetal MRI is often performed in fetuses with BPM, the aim of our study is to correlate CVR measurements by MRI and US, to compare inter-observer agreement of CVR measured by area-based MRI volume, diameter-based volume and to assess whether large lesions would be prone to more variability in CVRs among observers. Read More

Authors:  Emerson Miriam , Barth Richard , Rubesova Erika , Halabi Safwan , Rosenberg Jarrett , Hintz Susan , Blumenfeld Yair , Girsen Anna , Neves Stephanie , Homeyer Margaret

Keywords:  Bronchopulmonary malformation, CVR, lung

Guo Chen,  Wang Qian,  Hu Li-wei,  Zhong Yu-min

Final Pr. ID: Poster #: SCI-004

The purpose of this study was to demonstrate manifestations of MSCT in Abernethy malformation and its diagnostic value. Read More

Authors:  Guo Chen , Wang Qian , Hu Li-wei , Zhong Yu-min

Keywords:  Abernethy malformation, Angiograph, Tomography

Rajderkar Dhanashree,  Sharma Priya,  Verma Nupur

Final Pr. ID: Poster #: EDU-133

GI Emergencies and congenital malformation in the neonate are variable in their presentation and can be seen from the hypo-pharynx to the anus. The pediatric radiologist often plays a key role in diagnosis and planning of early surgical management. Read More

Authors:  Rajderkar Dhanashree , Sharma Priya , Verma Nupur

Keywords:  Congenital, GI malformations, Newborn bowel obstruction, New born GI emergencies, GI congenital emergencies

Jordan Gregory,  Zavaletta Vaz,  Malone Ladonna,  Katz Danielle,  Nakano Taizo,  Kulungowski Ann,  Annam Aparna

Final Pr. ID: Poster #: EDU-051

Generalized Lymphatic Anomaly (GLA) is a rare multisystem congenital disorder originating from the abnormal development of the lymphatic system which occur under the spectrum of Complex Lymphatic Anomaly (CLA). In addition to GLA, other CLAs include Kaposiform Lymphatic Anomaly (KLA) and Gorham-Stout Disease (GSD). Lymphatic malformations (LM) associated with GLA are usually apparent at birth or by two years of age. GLA can affect almost any organ of the body but is most commonly associated with lymphatic abnormalities in the skin, abdominal/thoracic viscera and bone.

Multisite soft tissue LM can occur in all CLAs, with macrocystic lymphatic malformations being most common in GLA. These lesions can be found in the mediastinum, retroperitoneum, and subcutaneous tissue. Abdominal viscera involved include the spleen and liver. The frequency of focal splenic lesions is higher with GLA and KLA in comparison to GSD. On MRI, the lesions exhibit marked T2 hyperintensity with no discernable enhancement. In patients with larger splenic lesions, areas of T1 hyperintensity have been documented. Liver lesions in GLA have a similar appearance to the previously described splenic lesions. Nakamura et al. found that more than 30 focal splenic lesions and/or focal splenic lesions with maximum diameters greater than >10 mm were observed only in patients with GLA. On contrast enhanced CT, the lesions are generally well-circumscribed and hypodense. Within the thorax, mediastinal LMs can be seen in GLA but are more common in KLA and GSD. Chylous effusions can occur in all of the CLAs, although it has been reported that effusions in GLA were more likely to be associated with mediastinal involvement. Osseus involvement is common in patients with GLA. GLA has a predilection for the appendicular skeleton, in which the ribs are most affected although cranial, vertebral and lower extremity lesions have been reported. The lesions are usually non-contiguous with medullary destruction and sparing of the cortex. This is in contrast to GSD where cortical destruction, progressive osteolysis, contiguous lesions, and soft tissue infiltration are more common.

Educational Goals:
1) Illustrate the most common imaging characteristics by each organ system affected by GLA and how to differentiate GLA from other CLAs.
2) Raise awareness of the optimal imaging evaluation in patients with GLA.
3) Outline an approach to multidisciplinary management of patients with GLA through a vascular anomalies center. Read More

Authors:  Jordan Gregory , Zavaletta Vaz , Malone Ladonna , Katz Danielle , Nakano Taizo , Kulungowski Ann , Annam Aparna

Keywords:  Generalized Lymphatic Anomaly, Lymphatic Malformations, Complex Lymphatic Anomaly

Rodríguez Garza Claudia,  Guillen Gutierrez Cinthia,  Elizondo Riojas Guillermo

Final Pr. ID: Poster #: EDU-064

Review the causes of cerebral hemorrhage in the pediatric patient based on clinical cases.

Stroke is a major cause of morbidity and mortality in children worldwide. The reported annual incidence ranges from 2.3 to 13 per 100,000 children per year in developed countries. In contrast to the adult, in children, diagnosis is not as straightforward, because when children present with acute neurological deficits, stroke is often not the first diagnosis considered by the medical providers.

Neuroimaging is essential for diagnosis and differentiation of stroke from stroke mimics that can present similarly.

Stroke in children can be ischemic or hemorrhagic, referring to the term hemorrhagic stroke as a intracerebral hemorrhage that is nontraumatic and whose most common etiology is secondary to a vascular malformation (Arteriovenous Malformation (AVM), Dural/Pial Arteriovenous Fistula (Dural AVF/Pial AVF), Cavernous Malformation, Vein of Galen Aneurysmal Malformation (VGAM), Developmental Venous Anomaly (DVA), Capillary telangiectasia, Sinus Pericranii, Aneurysms).

The most commonly used classification of vascular malformation is based on angioarchitectural and histomorphological characteristics. To differentiate these classic types, in a first step, shunting lesions have to be discerned from nonshunting lesions, as well as other features of the vascular contribution that will be evaluated by image.

We cannot forget the hemorrhage associated with coagulopathy, haematological disorders, brain tumors and cerebral sinovenous thrombosis (CSVT) which in the latter case can be found as venous infarction or hemorrhage; and other less common causes of hemorraghe in pediatrics as the intracranial aneurysms, in 15% of all pediatric aneurysms are secondary to an infection (micotic aneurysm).

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Authors:  Rodríguez Garza Claudia , Guillen Gutierrez Cinthia , Elizondo Riojas Guillermo

Keywords:  vascular malformations, hemorrhage, pediatric stroke

Shah Jignesh,  Chauhan Ankita,  Sandhu Preet

Final Pr. ID: Poster #: EDU-082

1. To discuss etiologies of unilateral hyperlucent hemithorax in children.
2. To discuss radiographic and cross-sectional imaging features of various conditions leading to unilateral hyperlucent hemithorax. Read More

Authors:  Shah Jignesh , Chauhan Ankita , Sandhu Preet

Keywords:  Unilateral hyperlucent hemithorax, congenital lobar overinflation, congenital pulmonary airway malformation

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

Jain Nikshita,  Haider Seemab,  Boag Graham,  Bhandal Samarjeet

Final Pr. ID: Poster #: EDU-019

Anorectal malformations are a fairly common group of congenital anomalies presenting at birth and often as part of a syndrome, with various other associated entities. The purpose of this article is to detail the role of imaging in the evaluation of the anomalies and provide a simple approach to reporting that will provide all the necessary anatomic details to the surgeons for best possible surgical and functional outcome. Read More

Authors:  Jain Nikshita , Haider Seemab , Boag Graham , Bhandal Samarjeet

Keywords:  Anorectal, congenital, malformations

Elmanzalawy Alaa,  Wei Xing-chang,  Ortiz-neira Clara,  Al Shuaili Ibtisam,  Bhandal Samarjeet

Final Pr. ID: Poster #: EDU-060

Review CT and MR imaging protocols in imaging children with sensorineural hearing loss.
Review normal anatomy of the inner ear.
Discuss the role of CT and MR imaging in the evaluation of children with sensorineural hearing loss.
Describe CT and MR imaging features of congenital inner ear malformations; with emphasis on what radiologists need to include in their report for patients considered for cochlear implantation. Read More

Authors:  Elmanzalawy Alaa , Wei Xing-chang , Ortiz-neira Clara , Al Shuaili Ibtisam , Bhandal Samarjeet

Keywords:  inner ear malformations, sensorineural hearing loss, cochlear malformation

Patel Nimai,  Swana Hubert,  Johnson Craig

Final Pr. ID: Poster #: SCI-023

To assess the incidence, scope, clinical findings and imaging characteristics of GU pathology due to underlying KTS in pediatric patients with the goal of improved diagnosis and outcomes for children with this potentially fatal disorder. Read More

Authors:  Patel Nimai , Swana Hubert , Johnson Craig

Keywords:  Klippel-Trenaunay syndrome, lymphatic malformation, Venous malformation

Dance Logan,  Keehn Brian,  Patel Mittun,  Pokorney Amber,  Peterson Michael,  Aria David,  Barnes Craig,  Bailey Smita

Final Pr. ID: Poster #: SCI-034

3D mDIXON Gradient Echo (GRE) technical superiority has already been established: improved fat saturation, faster acquisition time, high spatial resolution, and volumetric data acquisition allowing for a comprehensive multiplanar and 3D post-contrast evaluation of vessels and soft tissue lesions. Vascular malformation imaging requires large field of view images that can adversely affect fat saturation, is frequently performed with a non-sedated patient resulting in motion artifact, and requires higher spatial resolution to better characterize a lesion and evaluate lesion extent. Additionally the interventional radiologists at our institution prefer 3D post-contrast imaging to assist in treatment planning. We believe that this sequence is well suited for vascular malformation MRI imaging. The primary purpose is to illustrate the clinical utility and superior image quality of the 3D mDIXON GRE sequence over more conventional mDIXON TSE sequence through a series of cases of a variety of vascular malformations in different body regions. The secondary purpose of this presentation is to validate the clinical superiority of this sequence through a blinded reviewer comparison to the more conventional mDIXON TSE sequence. Read More

Authors:  Dance Logan , Keehn Brian , Patel Mittun , Pokorney Amber , Peterson Michael , Aria David , Barnes Craig , Bailey Smita

Keywords:  vascular malformation, MRI, water fat imaging

Ganapathy Shankar,  Hunter Kyle,  Janitz Emily,  Sreedher Gayathri

Final Pr. ID: Poster #: EDU-017

Neuronal migration and cortical organization takes place primarily during the second trimester and early third trimester. Therefore, detection of cortical malformations and migrational anomalies on a fetal MRI in a maturing brain is difficult and may not be evident in some cases, if performed early.
Familiarity with normal sulcation pattern in a fetus at various ages of gestation and a basic understanding of the embryogenesis of neuronal migration is essential to be able to make a diagnosis of these entities.

Goals of the exhibit
-Understand development of cerebral cortex and normal fetal MRI appearance at various stages of gestation.
- Using this knowledge, be able to recognize various cortical malformations in a fetus and provide a comprehensive diagnosis regarding etiology, whenever feasible or when the appearance is typical or when there are ancillary findings
- Avoid certain pitfalls and improve accuracy without overcalling or undercalling migrational anomalies in a fetal MRI

Outline of the exhibit
Embryology of neuronal migration (during second and third trimesters)
Normal sulcation pattern during mid and late trimesters
Etiology of neuronal migration and cortical malformations
-Multifactorial
- Infectious
- Genetic mutation/chromosomal
- Ischemic insult
- Metabolic disorders like Zellweger
Appearance of various migrational anomalies and cortical malformations in a fetal brain
Polymicrogyria – subtle and extensive forms, appearance during various stages of gestation, correlation with post-natal imaging, typical appearance in certain genetic mutations, TORCH infections etc
Schizencephaly – associations with absence of septum pellucidum and other abnormalities
Gray matter heterotopia – How to differentiate from subependymal nodules and hemorrhage?
Lissencephaly – How to identify a true lissencephaly/pachygyria in a developing brain with immature sulcal pattern?
Hemimegalencephaly, Microcephaly with simplified gyral pattern and other cortical malformations
Evolution of cortical malformations on imaging
-Comparison between early and late gestation fetal MRIs in the same patient when available
- Prenatal and post-natal MRI correlation of migrational anomalies, when available
Ancillary CNS and extra-CNS abnormalities identified in fetuses with migrational anomalies
Pitfalls and false positive/ false negative cases and how to avoid them.
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Authors:  Ganapathy Shankar , Hunter Kyle , Janitz Emily , Sreedher Gayathri

Keywords:  migrational anomalies, cortical malformations

Vegunta Aishwariya

Final Pr. ID: Poster #: EDU-037

A pictorial review of imaging appearances of anatomic variations of the portal vein.
Classification and pictorial review of various congenital abnormalities of the portal vein.
Common variations and branching patterns include trifurcation of the main PV, right posterior segmental branch arising from the main PV, and right anterior segmental branch arising from the left PV. Other less common branching patterns include left PV arising from the right anterior segmental branch, duplication of PV and absent branching of PV.
Portal venous shunts may be either portosystemic shunts with abnormal communication between the portal and systemic venous systems or arterioportal shunts between portal venous system and hepatic artery.
Portosystemic shunts bypass the liver and shunt directly portal venous blood into systemic venous system.
Congenital intrahepatic portosystemic shunts are classified into four types based on Park et al. Type I, the most common type, a single vessel connects the right portal vein to the IVC. Type II in which peripheral shunt localized to one hepatic segment and single or multiple communications between branches of portal and hepatic veins. Type III with aneurysmal connection between portal and hepatic veins. Type IV with multiple communications between peripheral portal and hepatic veins in several segments.
Abernethy malformations/congenital extrahepatic portosystemic shunts can be divided into 2 major types. Type I with complete absence of intrahepatic portal venous flow and complete shunting. Type Ia with congenital absence of the portal vein and separate drainage of superior mesenteric vein and splenic vein into systemic veins. Type IIb in which superior mesenteric vein and splenic vein join to form a short extrahepatic portal vein which drains into a systemic vein. Type II with some preserved hepatic portal venous flow and partial shunting. Type IIa arising from left or right portal vein including patent ductus venosus. Type IIb arising from main portal vein. Type IIc arising from the other portal vein tributaries. Patients with both these types suffer premature mortality either due to shunting complications or other associated congenital abnormalities.
Congenital arterioportal shunts may present either as a fistula or as vascular malformations in hereditary hemorrhagic telangiectasia.
Aneurysms of the PV may represent approximately 3% of all aneurysms of the venous system. Read More

Authors:  Vegunta Aishwariya

Keywords:  Portal vein, Congenital intrahepatic portosystemic shunts, Abernethy malformations/congenital extrahepatic portosystemic shunts

Goncalves Luís,  Jain Shilpa,  Krishnan Ananth,  Yuxiang Zhou,  Bloom David,  Lee Wesley,  Romero Roberto

Final Pr. ID: Poster #: SCI-079

To develop reference ranges for cerebellum and posterior fossa measurements by fetal MRI and to investigate the reproducibility and diagnostic performance of quantitative MR parameters for the differentiation of fetal posterior fossa abnormalities. Read More

Authors:  Goncalves Luís , Jain Shilpa , Krishnan Ananth , Yuxiang Zhou , Bloom David , Lee Wesley , Romero Roberto

Keywords:  posterior fossa, cerebellar vermis, Blake's pouch cyst, fetal MRI, Dandy-Walker malformation

Berger Jonathan,  Thomas Anna,  Teague Clint

Final Pr. ID: Poster #: CR-052

* To present a rare case of rectosigmoid venous malformation in a 15-year old male patient without Klippel Trenaunay Syndrome.
* To review common syndromic and nonsyndromic causes of pediatric lower gastrointestinal bleeding with presentation of relevant imaging.
* To discuss the classic imaging features and various treatment options for colorectal venous malformations. Read More

Authors:  Berger Jonathan , Thomas Anna , Teague Clint

Keywords:  lower gastrointestinal bleeding, vascular malformations of the lower GI tract

Ricci Kiersten,  Mobberley-schuman Paula,  Hammill Adrienne,  Dasgupta Roshni,  Patel Manish

Final Pr. ID: Poster #: SCI-031

Slow-flow vascular malformations, most commonly multifocal or diffuse venous (VM), venous-lymphatic (VLM) and capillary-lymphatic-venous malformations (CLVM), are associated with coagulation abnormalities affecting hemostasis and thrombosis and increase risk of hematological complications with procedural interventions. Although not completely understood, pathogenesis of this coagulopathy, termed localized intravascular coagulopathy (LIC), is presumed secondary to stagnant blood in abnormal vessels and consumption of coagulation factors. LIC is characterized by elevated D-dimer, low fibrinogen, and/or mild thrombocytopenia and may progress to disseminated intravascular coagulopathy following surgical procedures. In patients with high-risk malformations, hematologic complications of sclerotherapy and use of low molecular weight heparin (LMWH) as a preventative measure have not been well studied. Read More

Authors:  Ricci Kiersten , Mobberley-schuman Paula , Hammill Adrienne , Dasgupta Roshni , Patel Manish

Keywords:  LIC, Vasculat Malformation, Coagulopathy