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

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

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

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

Izhar Zain

Final Pr. ID: Poster #: CR-039

An 8-week-old male, born without complication at 33 weeks gestation age, presented with an undifferentiated posterior-midline scalp mass that had been present since birth. According to the mother, the mass has progressively increased in size with subjective tenderness to palpation. The mother reported possibly relevant two seizure-like episodes around 5 weeks of age consisting of jerking leg movements and staring spells with rapid blinking. Otherwise, the infant was healthy without any documented past medical or surgical history. Based on the presentation, a dedicated sonogram of the head was ordered with the goal of visualizing the mass and underlying cranial anatomy. The mass was demonstrated to be extracranial with avid vascularity. A few images raised suspicion for a trans-osseous vascular connection between the mass to the sub-adjacent dural venous sinuses. Subsequent contrast enhanced MRI and MRV imaging was ordered to distinguish the leading differential of Sinus Pericranii and Atretic Parietal Cephalocele. On MRI, the highly vascular extracranial mass demonstrated enhancement pattern similar to the dural venous sinuses. A trans-ossoeus vascular channel was seen arising from the mass coursing intracranially and communicating with the superior sagittal sinus. No arterial channel was visualized. No evidence of a persistent falcine venous sinus, primitive falcine vein or CSF tract was visualized. Subsequently, the diagnosis of Sinus Pericranii was established.

Sinus Pericranii and Atretic Parietal Cephalocele are extracranial subscapular masses that present similarly on physical examination and overall presentation. Sinus Pericranii is a low flow vascular malformation with an abnormal connection to the dural sinuses. Atretic parietal cephaloceles are small subscapular lesions with dura, dysplastic brain and fibrous tissue associated with variant intracranial anatomy including a persistent falcine venous sinus/primitive vein and/or abnormal CSF tract. Subsequently imaging findings are essential in distinguishing between the two entities and arriving at a definitive diagnosis. The presented case demonstrates this scenario. Read More

Authors:  Izhar Zain

Keywords:  Sinus Pericranii, Atretic Parietal Cephalocele, Vascular Malformation

Chern Joshua,  Mallon Mea,  Urbine Jaqueline,  Malik Archana,  Kazmi Faaiza,  Poletto Erica,  Faerber Eric

Final Pr. ID: Poster #: EDU-095

Soft tissue vascular malformations encompass a wide variety of lesions throughout the body. Appropriate treatment for these lesions is dependant on accurate classification and diagnosis. Unfortunately, inappropriate nomenclature and description by the radiologist can lead to confusion and possibly mismanagement. Read More

Authors:  Chern Joshua , Mallon Mea , Urbine Jaqueline , Malik Archana , Kazmi Faaiza , Poletto Erica , Faerber Eric

Keywords:  Arteriovenous malformation, Vascular malformation, Lymphatic malformation, Vascular tumor

Sams Cassandra,  Ayyala Rama

Final Pr. ID: Poster #: CR-014

Mesenteric lymphatic malformations are rare intra abdominal masses. Large mesenteric malformations can present soon after birth secondary to abdominal distention or failure to thrive. However, they can also remain clinically inapparent throughout childhood. In this series, three patients, ranging in age from 8 to 16 years, present with acute or acute on chronic abdominal pain. Subsequent imaging studies demonstrated macrocystic, mesenteric lymphatic malformations. These malformations may become painful in the setting of hemorrhage or superimposed infection. As this condition is likely not at the forefront of the clinician's mind, it is incumbent upon the radiologist to recognize the imaging findings. This case study will present a multimodality approach to the diagnosis of macrocystic, mesenteric lymphatic malformations. Read More

Authors:  Sams Cassandra , Ayyala Rama

Keywords:  Vascular Malformation, Abdominal

Golden Eleza,  Kadom Nadja

Final Pr. ID: Poster #: CR-039

On conventional MRI pediatric vasoproliferative neoplasms or vascular malformations may demonstrate similar features including T2 hyperintensity and enhancement. Hemangiomas can appear similar to vascular malformations when there are large feeding vessels.
Our objective is to illustrate typical imaging findings of vasolproliferative neoplasms and vascular malformations of the head and neck on conventional MRI and the benefits of time resolved MRA to further distinguish between high and slow flow lesions Read More

Authors:  Golden Eleza , Kadom Nadja

Keywords:  time resolved MRA, vascular malformation