Final Pr. ID: Poster #: EDU-073
The purpose of educational exhibit is to summarize the radiological appearances of various conditions causing basal ganglia (BG) calcification in children. The pathogenesis for symmetric BG calcification is diverse and ranges from benign physiological calcifications to a variety of pathological disorders including metabolic, infectious and genetic diseases. We present a practical approach to further narrow the differential diagnosis based on associated musculoskeletal imaging findings in patients with BG calcification.
Parathyroid disorders are the most common causes of pathological BG calcification. In hyperparathyroidism, distinctive subperiosteal bone resorption can be seen in phalanges on hand radiographs. Tapering of the clavicles, brown tumors and salt-and-pepper appearance of the skull are also classic radiographic features. In the Albright hereditary osteodystrophy phenotype of pseudohypoparathyroidism, shortening of the fourth and fifth metacarpals as well as advanced bone age can aid in diagnosis. Tumoral calcinosis is another radiographically distinct disease that can cause BG calcification, characterized in the extremities by periarticular calcific deposits. Cockayne syndrome is a rare autosomal recessive (AR) disorder with 4 overlapping subtypes, all with BG calcification as well as diffuse skeletal abnormalities that become apparent in the toddler years. Carbonic anhydrase deficiency type 2 is another rare AR disorder with intracranial calcification including the BG, as well as osteopetrosis and pathologic fractures. Coat’s plus syndrome has characteristic rock-like intracranial calcifications paired with leukodystrophy and brain cysts. It manifests skeletally with osteopenia, delayed fracture healing, bowing of long bones, scoliosis, midface hypoplasia and femoral head avascular necrosis. Other common conditions such a Down syndrome and HIV, and rare disorders such Fahr's disease, malignant phenylketonuria, and Aicardi-Goutieres syndrome are in the differential diagnosis of BG calcifications. Read More
Final Pr. ID: Poster #: SCI-012
Perinatal Hypoxic Ischemic Injury (HII) has a higher prevalence in the developing world. One of the primary concepts for suggesting that an imaging pattern reflects a global insult to the brain is when the injury is noted to be bilateral and symmetric in distribution. In the context of HII in term neonates, this is either bilateral symmetric (a) peripheral/watershed injury (WS) or (b) bilateral symmetric Basal-ganglia-thalamus (BGT) pattern, often with the peri-Rolandic and hippocampal injury. Unilateral, asymmetric or unequal distribution of injury may therefore be misdiagnosed as perinatal arterial ischemic stroke. This has been previously reported in the typical distribution patterns, without a clear indication of the prevalence. We aimed to determine the prevalence of unilateral, asymmetric and unequal degree HII and to characterize the distribution on MRI in a large database of patients with Cerebral Palsy (CP) from South Africa. Read More