Hu Anna,  Kim Jane,  Peck Jeffrey,  Haroyan Harutyun

Final Pr. ID: Poster #: EDU-059

Idiopathic hip chondrolysis (ICH) is a rare pediatric musculoskeletal disorder characterized by rapid and extensive loss of articular cartilage at the femoral head and acetabulum. Symptoms of ICH, such as pain, stiffness, and limping, are often nonspecific and may overlap with other pathologies. Diagnosis of ICH is multifactorial and relies on the correlation of imaging with the clinical presentation. Due to both the rarity of this entity and nonspecific clinical symptoms, ICH may be underdiagnosed.
In this educational poster, we will review the clinical presentation, describe the imaging findings of ICH with a special emphasis on MRI features using examples from our own institution, and discuss the treatment options as well as the natural progression of disease. Recognition of the imaging features is vital in early identification of ICH which may help prevent further irreversible cartilage loss. Read More

Authors:  Hu Anna , Kim Jane , Peck Jeffrey , Haroyan Harutyun

Keywords:  idiopathic, hip chondrolysis, MRI

Panwar Jyoti,  Kirkhus Eva,  Meyers Arthur,  Patel Hiten,  Van Rossum Marion,  Stimec Jennifer,  Tse Shirley,  Twilt Marinka,  Tzaribachev Nikolay,  Doria Andrea,  Tolend Mirkamal,  Akikusa Jonathan,  Herregods Nele,  Highmore Kerri,  Inarejos Clemente Emilio,  Jans Lennart,  Jaremko Jacob Lester,  Von Kalle Thekla

Final Pr. ID: Poster #: SCI-036

Whole body (WB) MRI is a valuable method for surveying the overall burden of systemic diseases such as juvenile idiopathic arthritis (JIA), directing further diagnostic and treatment approaches. Consensus-driven development and validation of a standardized WB-MRI scoring system for JIA has important clinical utility in timely detection and monitoring of disease activity, and serves as an outcome measure in research. We describe our experience utilizing a formal consensus approach amongst imaging and/or clinical JIA experts towards developing a novel WB MRI scoring system to assess disease activity in JIA. Read More

Authors:  Panwar Jyoti , Kirkhus Eva , Meyers Arthur , Patel Hiten , Van Rossum Marion , Stimec Jennifer , Tse Shirley , Twilt Marinka , Tzaribachev Nikolay , Doria Andrea , Tolend Mirkamal , Akikusa Jonathan , Herregods Nele , Highmore Kerri , Inarejos Clemente Emilio , Jans Lennart , Jaremko Jacob Lester , Von Kalle Thekla

Keywords:  Consensus, WB-MRI scoring system, Juvenile idiopathic arthritis

Junhasavasdikul Thitiporn,  Kellenberger Christian,  Tolend Mirkamal,  Doria Andrea

Final Pr. ID: Poster #: EDU-107

Juvenile Idiopathic Arthritis (JIA) is the most common musculoskeletal disorder in children. The temporomandibular joints (TMJs) are involved in majority of cases, and detection of early arthritic changes in these joints relies on contrast enhanced magnetic resonance imaging (MRI). However, with the paucity of evidence and consensus on the normal and pathologic changes in this small but complex joint, interpretation of TMJ MRIs remains to be difficult. This exhibit presents the normal growth related and arthritis related variations in the disease progression in TMJ MRIs of JIA patients. Read More

Authors:  Junhasavasdikul Thitiporn , Kellenberger Christian , Tolend Mirkamal , Doria Andrea

Keywords:  Temporomandibular joint, Juvenile idiopathic arthritis, Magnetic resonance imaging atlas

Fontalvo Herazo Lucia,  Putnins Rita,  Rotaru Carmen

Final Pr. ID: Poster #: CR-011

Idiopathic arterial calcification of infancy (IACI) is a rare condition, characterized by extensive depositions of hydroxyapatite in the internal elastic lamina of medium-sized and large arteries. It is usually diagnosed prenatally or in early infancy. Congenital intrahepatic portosystemic shunt is a persistent communication between the vitelline veins and sinus venosus, due to a focal absence of sinusoid formation. Infrequently they can present with signs of portal hypertension such as varices, ascites, and splenomegaly, but in most cases this feature usually indicates that the existing shunt is compensatory and not congenital.

We present a 29 week male neonate with a prenatal sonographic examination showing pericardial effusion, severe ascites and calcified cardiac outflow tract. After C section, the patient required resuscitation, intubation and abdominal drainage due to ascites in the NICU. Postnatal abdominal ultrasound confirmed abnormal calcification of the wall of the abdominal aorta, common iliac and origin of the superior mesenteric artery. Cranial ultrasound showed abnormal heterogeneous echotexture of the brain parenchyma with several linear parallel echogenicities, that may correspond to microcalcification of small caliber arteries. During the first few days of life the patient developed liver failure, severe cholestasis, coagulopathy and hypoalbuminemia. Follow up ultrasound done on day 12 showed hepatosplenomegaly, multiple hypoechoic focal liver lesions and an intrahepatic portosystemic shunt between the bifurcation of the main portal vein and the left hepatic vein. This finding was determined to be the primary cause of the ascites and progressive hepatosplenomegaly. By day 65 the portosystemic shunt had spontaneously resolved. Unfortunately the idiopathic arterial calcification had significantly progressed, despite bisphosphonate treatment. Multisystem organ failure had developed, the patient died on day 68.

DNA sequencing and high density target array analysis of ABCC6 and ENPP1 revealed no detectable mutation (anomaly most frequently related with IACI). Nevertheless, a c2320C>T transition in exon 23ENPP1 was identified. Finding been previously reported in a case of periarticular calcification.

This case illustrates two different and important pathologies that can be present in neonates. Each one has specific features and significant clinical relevance. This is a rare association as these two entities are usually seen separately. Read More

Authors:  Fontalvo Herazo Lucia , Putnins Rita , Rotaru Carmen

Keywords:  Idiopathic arterial calcification, Transient congenital porto systemic shunt, Peterme baby

Otobo Tarimobo,  Rumsy Dax,  Inarejos Emilio,  Kirkhus Eva,  Stimec Jennifer,  Panwar Jyoti,  Thorpe Kevin,  Jans Lennart,  Van Rossum Marion,  Tolend Mirkamal,  Perez Manuela,  Conaghan Philip,  Tzaribachev Nikolay,  Sandhya Pulukool,  Tse Shirley,  Simone Appenzeller,  Swami Vimarsha,  Touma Zahi,  Lambert Robert,  Doria Andrea,  Maksymowych Walter,  Van Der Heijde Desiree,  Weiss Pamela,  Sudol-szopinska Iwona,  Herregods Nele,  Jaremko Jacob,  Meyers Arthur

Final Pr. ID: Poster #: EDU-061

Clinical assessment of the Sacroiliac Joint (SIJ) is limited due to the location and anatomy of the joint. Magnetic Resonance Imaging is a sensitive, non- invasive tool in detecting early SIJ inflammatory changes and structural damage in Juvenile Idiopathic Arthritis (JIA). The quantification of interval change of pediatric SIJs using MRI based scoring methods will serve as an important objective outcome measure for the assessment of disease severity and treatment effectiveness in JIA. Read More

Authors:  Otobo Tarimobo , Rumsy Dax , Inarejos Emilio , Kirkhus Eva , Stimec Jennifer , Panwar Jyoti , Thorpe Kevin , Jans Lennart , Van Rossum Marion , Tolend Mirkamal , Perez Manuela , Conaghan Philip , Tzaribachev Nikolay , Sandhya Pulukool , Tse Shirley , Simone Appenzeller , Swami Vimarsha , Touma Zahi , Lambert Robert , Doria Andrea , Maksymowych Walter , Van Der Heijde Desiree , Weiss Pamela , Sudol-szopinska Iwona , Herregods Nele , Jaremko Jacob , Meyers Arthur

Keywords:  Juvenile idiopathic arthritis, OMERACT, outcome measure