Henry M Katherine,  White Ammie,  Servaes Sabah,  Wood Joanne

Final Pr. ID: Poster #: EDU-031

Infants are at risk for fatal physical abuse yet may present to care multiple times with inflicted trauma before abuse is diagnosed and the infant is protected. Prompt identification of at-risk infants is essential to protection from ongoing abuse. Sentinel injuries are unexplained minor injuries in young infants that are appreciable on physical exam. Examples of sentinel injuries include bruising, subconjunctival hemorrhages, and intra-oral trauma. These injuries can be a subtle external sign of concurrent clinically occult internal injuries (e.g., skeletal, abdominal, intracranial injuries) in a well-appearing infant or may be an early warning that foretells more significant trauma in the future. Identification and evaluation of these infants with appropriate imaging has been highlighted as one approach to ensure that abuse is promptly detected, injuries are addressed, and the infant is protected.

In this multidisciplinary educational presentation made in collaboration with child abuse pediatricians and pediatric radiologists, we will: (1) review different types of sentinel injuries; (2) discuss current evidence and guidance for performance of imaging (skeletal survey, abdominal CT, neuroimaging with MRI or CT) when a sentinel injury is identified; (3) discuss the interpretation and significance of imaging findings; (4) provide imaging examples of these injuries; and (5) discuss gaps in the literature while highlighting opportunities for collaboration between child abuse pediatricians and pediatric radiologists to improve the care of these infants.

Following completion of this educational module, our goals are that the participant will better understand the clinical context of sentinel injuries and have a baseline knowledge to make an appropriate imaging plan for these infants. Read More

Authors:  Henry M Katherine , White Ammie , Servaes Sabah , Wood Joanne

Keywords:  Child Abuse, Sentinel Injuries, Skeletal Survey

Do Patrick,  Krishnarao Priya

Final Pr. ID: Poster #: EDU-103

Assessing and reporting pediatric bone age is a subjective and often tedious multi-step process. The typical reporting workflow at our institution requires perusing a hardcopy of a bone age atlas to find an image matching the PACS screen, finding reference values in a table, and performing rote arithmetic to determine the progress of skeletal maturity. Read More

Authors:  Do Patrick , Krishnarao Priya

Keywords:  bone, age, skeletal, structured, report

Loomis Judyta,  Bulas Dorothy,  Rubio Eva,  Blask Anna

Final Pr. ID: Poster #: EDU-112

The purpose of this case series is to describe the variable prenatal presentation of lethal and nonlethal hypophosphatasia, obstetrical outcomes, and postnatal course of the nonlethal cases.

5 genetically proven cases of hypophosphatasia were evaluated in our fetal imaging center from 2009 to 2016 and initially imaged between 17 weeks and 34 weeks gestation. The prenatal imaging, prenatal testing, pregnancy outcome, and postnatal or autopsy imaging and genetic testing are reviewed in this case series.

All 5 cases were referred with a high suspicion of a skeletal dysplasia. Fetal sonography demonstrated a spectrum of bowing, shortening, and acute fractures of the long bones, variable involvement of the upper and lower extremities, and axial skeleton involvement.
Of the 5 cases, 3 were nonlethal. Postnatal imaging in these cases was concordant with the prenatal assessment of variable mild limb shortening and bowing and without involvement of the axial skeleton.
Case 4 was terminated at 20 weeks due to severe micromelia and irregular long bones with postmortem radiographs demonstrating shortening and fractures with severe demineralization of the skull.
Case 5, first evaluated at 29 weeks gestation demonstrated severe bowing and shortening of the long bones, a small chest circumference with beading of the ribs. Postnatal radiographs demonstrated spurs in the midshafts of the fibula and ulna with severely demineralized skull. The infant died in the immediate postnatal period. Read More

Authors:  Loomis Judyta , Bulas Dorothy , Rubio Eva , Blask Anna

Keywords:  dysplasia, Fetal, skeletal

Parnell Shawn

Final Pr. ID: Poster #: EDU-080

The skeletal dysplasias are a large diverse group of several hundred disorders which are marked by abnormal bone and cartilage growth with resultant short stature. Dysplasias have been divided into larger groups according to common radiographic and/or genetic mutations. The purpose of this educational exhibit is to highlight one of these major groups, which are characterized by mutations of type 2 collagen. Read More

Authors:  Parnell Shawn

Keywords:  skeletal dysplasia, musculoskeletal, type 2 collagen, skeletal survey, dwarfism

Aboagye Rosemond,  Bajno Lydia,  Nadel Helen,  Potts James,  Bray Heather

Final Pr. ID: Poster #: SCI-063

Purpose: ¹⁸F-FDG Positron Emission Tomography (PET-CT) has been shown to be superior to other imaging modalities in assessment of soft tissue involvement with Langerhans Cell Histiocytosis (LCH) and is now commonly included in the staging workup of LCH. Our purpose is to analyze the utility of skeletal survey in addition to PET-CT scan for detecting bone lesions in children with LCH and to evaluate if skeletal survey can be eliminated from the staging workup of LCH in order to reduce radiation exposure. Read More

Authors:  Aboagye Rosemond , Bajno Lydia , Nadel Helen , Potts James , Bray Heather

Keywords:  Langerhans Cell Histiocytosis, PET-CT, Skeletal Survey

Hwang Misun,  Servaes Sabah

Final Pr. ID: Poster #: EDU-088

Many normal variants in the skeletal survey can be misconceived as signs of child abuse. Accurate differentiation between the two is crucial in subjecting only the perpetrators to trial. The present exhibit educates on the radiographic signs and techniques that can help differentiate between normal variants and child abuse on the skeletal survey. Read More

Authors:  Hwang Misun , Servaes Sabah

Keywords:  Child abuse, Skeletal Survey, Normal variants

Marine Megan,  Smith Jessica,  Wanner Matthew,  Jennings Samuel,  Hibbard Roberta,  Karmazyn Boaz

Final Pr. ID: Poster #: SCI-030

The purpose is to evaluate how well hospitals comply with ACR-SPR guidelines for skeletal surveys in the cases of possible child abuse. Read More

Authors:  Marine Megan , Smith Jessica , Wanner Matthew , Jennings Samuel , Hibbard Roberta , Karmazyn Boaz

Keywords:  Skeletal Survey, Child Abuse, Nonaccidental Trauma

Paddock Michael,  Offiah Amaka,  Sprigg Alan

Final Pr. ID: Poster #: EDU-004

Recognising the skeletal manifestations of inflicted injury (II) in infants and young children is of crucial importance. There are specific fracture patterns that are highly suspicious of II and common differential diagnoses with which radiologists should be familiar. Read More

Authors:  Paddock Michael , Offiah Amaka , Sprigg Alan

Keywords:  Inflicted injury, Non-accidental injury, Physical abuse, Skeletal survey, Child abuse

Averill Lauren,  Tomatsu Shunji,  Theroux Mary

Final Pr. ID: Poster #: CR-044

Morquio A syndrome is an autosomal recessive lysosomal storage disorder characterized by skeletal dysplasia and progressive disability due to orthopedic complications, spinal cord compression and airway compromise. Although the bony changes and cervical spine instability have been well described in the radiology literature, the importance of imaging the airway in these patients has received scant attention. The purpose of this poster is to illustrate the progressive abnormality of the thoracic inlet and trachea seen in children and young adults with Morquio A syndrome.
The interplay of pectus carinatum, hypertrophied clavicular heads and upper thoracic kyphosis leads to bony narrowing of the thoracic inlet. Furthermore, glycosaminoglycan deposition degrades the structural integrity of the tracheal wall, creating a twisted and floppy airway. Additional crowding by a crossing tortuous right brachiocephalic artery and sometimes the thyroid gland contribute to progressive narrowing of the trachea at the thoracic inlet. Imbalance of growth between the skeleton and the airway and blood vessels may also play a role.
We present a series of patients with Morqiuo A syndrome, with multimodality imaging depicting the complex anatomy of the thoracic inlet contributing to airway compromise. Radiographs of the neck, chest or spine can suggest airway narrowing with a tilted hourglass shape of the trachea seen in the frontal projection; lateral views, though, are often limited. MRI of the cervical spine, frequently acquired to evaluate the craniocervical junction, also allows for assessment of the thoracic inlet including the trachea and crossing right brachiocephalic artery. CT angiogram of the chest can more clearly delineate vascular, bony and airway relationships in individuals with declining respiratory function or unexpected airway difficulty during anesthetic management. Three dimensional rendering and airway fly-through techniques may help guide anesthetic care and, in extreme cases, airway reconstruction. The imaging features of the thoracic inlet in this group of Morquio A patients are correlated with clinical phenotype, pulmonary function tests, and bronchoscopy when available. Read More

Authors:  Averill Lauren , Tomatsu Shunji , Theroux Mary

Keywords:  trachea, skeletal dysplasia, brachiocephalic artery

Parikh Ashishkumar,  Luo Yu,  Spottswood Stephanie

Final Pr. ID: Poster #: CR-036

First described by Rathbun in 1948, hypophosphatasia is an inherited metabolic disorder arising from the deficient activity of the tissue-nonspecific isoenzyme of alkaline phosphatase. There are several different types and varying clinical presentations of hypophosphatasia, characterized according to their age of onset by Fraser in 1957. In addition, the severity of the radiographic findings is inversely correlated with the age of presentation, with older patients presenting with less severe forms of the disease. Classically, the radiographic findings resemble rickets/osteomalacia, but in the presence of normal Vitamin D metabolism. Additional findings associated with hypophosphatasia are Bowdler spurs, which are transverse bony spurs in the radius, fibula and ulna and central lucencies or “punched out” lesions in the metaphysis, particularly of the knee (Case 1). In this case series of 3 patients, these characteristic radiographic features as depicted on skeletal surveys, along with their clinical manifestations, diagnostic criteria, management, treatment, and prognosis will be discussed. In particular, the evolution of radiographic changes with treatment in one patient will be assessed (Case 2). Read More

Authors:  Parikh Ashishkumar , Luo Yu , Spottswood Stephanie

Keywords:  hypophosphatasia, skeletal dysplasia, phosphate, hypophosphatemia, Bowdler

Ulikowska Ewelina

Final Pr. ID: Poster #: EDU-012 (T)

Langerhans Cell Histiocytosis (LCH), is a disorder that primarily affects children, but can affect individuals of all ages. Langerhans cells are cells that are responsible for regulating immune system in our bodies. They are mostly found in the skin, spleen, lymph nodes, liver and bone marrow. In patients who have LCH, these cells grow and multiply excessively. The abnormal growth of the Langerhans cells causes a formation of tumors called granulomas. LCH can affect different areas of the body: skin, nails, lymph nodes, gastrointestinal tract, central nervous system, pituitary and thyroid gland, liver, lungs and bones. The severity and symptoms of the disease vary in individual patients and are dependent on the organs and systems affected. Oftentimes, LCH can be found in multiple areas of the body and when that happens, the disease becomes a multisystem disease. The most common system affected by LCH, seen in about 80 % of individuals affected, is the skeletal system. Granulomas, which develop most commonly in the flat bones such as skull and long bones of arms and legs, cause sclerotic and lytic lesions that can in turn become the cause of pathologic fractures. Therefore it is crucial, to recognize the radiographic signs of skeletal manifestations of LCH. Radiography is the preliminary imaging of choice and skeletal surveys are oftentimes the best assessment of the status of LCH prior and post treatment. The purpose of this abstract is to describe radiographic appearances associated with Langerhans Cell Histiocytosis. In order to confirm the importance of follow up skeletal surveys, I will present cases and associated radiographs that show signs of LCH prior to treatment and post treatment. Read More

Authors:  Ulikowska Ewelina

Keywords:  Langerhans Cell Histiocytosis, Radiographic findings, Skeletal manifestations

Vasireddi Anil,  Bradley Helen,  Shah Amisha

Final Pr. ID: Paper #: 111

EOS^TM is a biplanar radiographic imaging system that can simultaneously acquire whole-body frontal and lateral standing radiographs with moving x-ray tubes and detectors. EOS has been shown to reduce radiation exposure and study time in comparison to standard digital radiography. While it has primarily found utility in evaluation of scoliosis, there has been limited study of EOS in performing pediatric skeletal surveys, which can be time-consuming and challenging given the age of the patient and the large number of images required. This study evaluates how implementing EOS in combination with digital radiography (DR) can reduce the number of images acquired and improve patient/family experience. Read More

Authors:  Vasireddi Anil , Bradley Helen , Shah Amisha

Keywords:  Skeletal Survey, EOS, Xray

Miyazaki Osamu,  Sawai Hideaki,  Yamada Takahiro,  Murotsuki Jun,  Horiuchi Tetsuya,  Nishimura Gen

Final Pr. ID: Poster #: SCI-014

Fetal CT has almost the same utility as a postnatal skeletal survey. Despite this benefit, the associated radiation exposure is disadvantageous and radiation dose reduction is mandatory. It is however impossible to measure the actual radiation dose to the fetus directly. Several previous reports have described the CT dose index (CTDI) volume and dose length product (DLP) as representing an imagined fetal dose. The actual fetal radiation dose needs to be confirmed using a phantom that practically corresponds to a pregnant woman. Read More

Authors:  Miyazaki Osamu , Sawai Hideaki , Yamada Takahiro , Murotsuki Jun , Horiuchi Tetsuya , Nishimura Gen

Keywords:  skeletal dysplasia, fetal CT, radiation dose

Handa Atsuhiko,  Nishimura Gen

Final Pr. ID: Poster #: EDU-034

Skeletal dysplasias encompass a heterogeneous group of over 400 disorders. They are individually rare, but collectively common with an approximate incidence of 1/5000; thus, radiologists occasionally encounter skeletal dysplasias in daily practice. However, many radiologists and trainees struggle with this topic because of the lack of proper resources.

A group of skeletal dysplasias that share similar radiological patterns has been grouped into a “skeletal dysplasia family” which generally have common pathogenesis. For example, a skeletal dysplasia family comprising mucopolysaccharidosis, oligosaccharidosis, and mucolipidosis all radiologically exhibit dysotosis multiplex, and all share an abnormality in lysosome dysfunction.

The beauty of this family concept is its simplicity and power. First, it allows a more systematic approach to the chaotic world of skeletal dysplasias – a stepwise approach, with the first step of general pattern recognition and categorization of a certain case into a certain family, and the second step of diagnosis based on more meticulous observations for subtle but distinctive radiological findings. Since major skeletal dysplasia families are limited in number, the radiologist can become familiar with their patterns. Second, radiographs can predict the presence of genetic mutations. Geneticists or pediatricians would appreciate the correct radiological diagnosis even in today’s genetics practice with advanced molecular techniques. Radiological diagnosis and genetics are complementary. Third, it may lead us to a more precise assessment of radiological findings. Shared findings among family members allow more accurate characterization of the severest end of the family which occasionally look similar to the one with different pathogenesis.

The purpose of this exhibit is to demonstrate the imaging characteristics of major skeletal dysplasia families and the stepwise approach to the diagnosis. We will review the classic and more recently established dysplasia families. In addition, we will review clinical and genetic features that help radiologists to participate in multidisciplinary care.

Introduction – Dysostosis multiplex family – FGFR3 (achondroplasia) family – COMP family – Type II collagenopathies – TRPV4 (metatropic dysplasia) family – Skeletal ribosomopathies – DTDST (diastrophic dysplasia) family – Linkeropathies – Filaminopathies A and B – Punctata group – Skeletal ciliopathies – Osteogenesis imperfecta group Read More

Authors:  Handa Atsuhiko , Nishimura Gen

Keywords:  Skeletal dysplasia

Birkemeier Krista,  Ward Erica,  Salman Sehar,  Blazovic Sarah,  Mallett Lea

Final Pr. ID: Paper #: 065

Imaging during the evaluation of suspected child abuse victims may be requested 24 hours a day, however, pediatric radiologists may not always be available to interpret these studies. Timely reporting is important for treatment and disposition in this vulnerable population. In addition, the accuracy of detection of subtle injuries is critical for patient safety. The current protocol at our institution consists of an immediate preliminary interpretation by an adult radiologist when a skeletal survey is obtained after hours, and final interpretation by one of three pediatric radiologists during normal business hours. Read More

Authors:  Birkemeier Krista , Ward Erica , Salman Sehar , Blazovic Sarah , Mallett Lea

Keywords:  Skeletal Survey, Discrepancy, Child Abuse

Marine Megan,  Smith Jessica,  Wanner Matthew,  Jennings Samuel,  Hibbard Roberta,  Karmazyn Boaz

Final Pr. ID: Poster #: SCI-029

The purpose is to evaluate how well non-pediatric radiologists interpret skeletal surveys in the cases of possible child abuse. Read More

Authors:  Marine Megan , Smith Jessica , Wanner Matthew , Jennings Samuel , Hibbard Roberta , Karmazyn Boaz

Keywords:  Skeletal Survey, Child Abuse, Nonaccidental Trauma