Proximal humeral epiphyseal fracture-separation is a rare fracture pattern in infants often associated with birth-related or non-accidental trauma, representing a Salter-Harris type I or type II fracture. Lack of a proximal humeral epiphyseal ossification center in most newborns or only subtle displacement of a small epiphyseal ossification center in older infants, makes this injury difficult to diagnose on plain radiographs, potentially leading to delayed diagnosis or misdiagnosis. Ultrasound and MRI are therefore useful imaging modalities in indeterminate cases. Clinical findings of infant proximal humeral epiphyseal fracture-separation such as shoulder swelling, tenderness, and decreased active motion, overlap with more common entities including clavicular fracture, brachial plexus injury and osteomyelitis. Radiographs are often the first diagnostic study ordered to evaluate these symptoms but are insensitive due to minimal ossification of the proximal humeral epiphysis. Radiographs may be normal or show subtle displacement of the epiphyseal ossification center, apparent joint space widening, small metaphyseal fracture fragments or displacement of the proximal humeral metaphysis in relation to the scapula. These findings can be misdiagnosed as shoulder dislocation or pseudosubluxation due to a joint effusion. Careful review of the medical record may elucidate a history of difficult delivery with shoulder dystocia or suspected shoulder trauma. Ultrasound and MRI are useful in indeterminate cases due to their ability to resolve the cartilaginous physis and proximal humeral epiphysis, and to resolve their relationship to the humeral shaft and cartilaginous labrum. Ultrasound has higher anatomic resolution and offers the flexibility to quickly image the asymptomatic contralateral shoulder and image in planes that best show the relationship between the non-ossified epiphysis and the humeral shaft. Doppler ultrasound demonstrates epiphyseal perfusion without the need for contrast administration, and serial ultrasound imaging can be used to evaluate healing and remodeling. MRI is more useful in evaluating cases where osteomyelitis and/or septic arthritis are being considered, or in cases of an inconclusive history suspicious for non-accidental trauma to evaluate for additional osseous and soft tissue injuries. Several examples of typical proximal humeral epiphyseal fracture-separations in infants will be presented and the relevant imaging findings discussed. Read More

Meeting name: SPR 2019 Annual Meeting & Postgraduate Course , 2019

Authors: Fenlon Edward, Degnan Andrew, Maddocks Alexis, Chen Susie, Jaramillo Diego

Keywords: Fracture, Infant, Trauma

Gaucher disease is an inherited metabolic disorder due deficiency of the lysosomal enzyme β-glucocerebrosidase that results in the accumulation of abnormal macrophages (“Gaucher cells”) within multiple organs, most conspicuously affecting the liver, spleen and bone marrow. As the diagnosis is increasingly made during childhood and young adulthood, pediatric radiologists should be familiar with imaging features of Gaucher disease and its complications. Visceromegaly consisting of hepatosplenomegaly is a hallmark of Gaucher disease and uniformly present in cohorts of pediatric patients. In addition, bone marrow involvement with ‘Erlenmeyer flasks’ have been long recognized as part of this disease, although the classic radiographic finding is not present until adulthood. Marrow involvement confers significant morbidity for these patients with pain, bone infarcts and pathologic fracture. Traditionally, imaging of disease severity has been based on hepatic and splenic visceral organ enlargement and/or qualitative assessment of bone marrow involvement. However, advances in the understanding of Gaucher disease and observations of elevated ferritin levels and increased risk of hepatic fibrosis emphasize the importance of more comprehensive assessment of liver involvement beyond simple enlargement. Moreover, quantitative MRI assessment of bone fat-fractions also may have a role in assessing marrow involvement. These methods of disease assessment are important in addressing management decisions regarding enzyme replacement and substrate reduction therapy. Read More

Meeting name: SPR 2019 Annual Meeting & Postgraduate Course , 2019

Authors: Degnan Andrew, Serai Suraj, Ho-fung Victor, Barrera Christian, Wang Dah-jyuu, Ahrens-nicklas Rebecca, Ficicioglu Can

Keywords: Gaucher disease, bone marrow, hepatic involvement