To date, a standard for CRD has not been established. Plain radiographs are a cost-effective, low-risk option for studying osseous structures in great detail. It is well known that bone age is superior to chronological age for determining biological and structural maturity; for decades, pediatricians have relied on plain films of the wrist and hand for assessing bone age in children (e.g. Greulich & Pyle Atlas, Tanner Whitehouse Method). However, some have proposed new approaches with greater accuracy and reliability across all ages and ethnic groups. Here, we present a new standardized radiographic dimension, CRD, as a tool to assess developmental maturity, which can be readily obtained from standard medical imaging platforms. Read More

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

Authors: Roberts Dustin, Chawla Soni

Keywords: capitular-radial distance, developmental maturity, CRD

Optic nerve aplasia (ONA) is a very rare developmental anomaly characterized by absence of optic nerve, retinal ganglion, and optic nerve vessels. The majority of reported ONA cases are unilateral. The rarer cases of bilateral ONA are often associated with additional brain anomalies. ONA is considered distinct from optic nerve hypoplasia. Both environmental and genetic factors have been hypothesized to contribute to ONA, and several genetic mutations have been identified, including PAX6, OTX2, CYP26A1, or CYP26C1. We report a case of bilateral ONA in an otherwise healthy infant with mild hypoplasia of the corpus callosum without dysplasia, and macrocephaly. A 13-month-old male presented for evaluation of macrocephaly and global developmental delay that was attributed to cortical blindness. He was born full-term without complications, however mother felt no fetal movement for one week prior to delivery. Abnormal roving eye movements were noted since the early neonatal period. There was no family history of any congenital diseases. Examination revealed conjugate non-purposeful rapid eye movements without nystagmus, bilateral lens opacification, and absence of the pupillary light response, startle reflex, and red reflex. The remainder of the physical exam was normal. MRI of the brain and obits demonstrated absence of the bilateral optic nerves, optic chiasm, and the optic tracts. The globes were normal in size and appearance, although with the right minimally larger than the left. The corpus callosum was relatively thin, but intact, suggesting mild hypoplasia without dysplasia. In contrast to the majority of reported bilateral ONA cases that have associated CNS anomalies (polymicrogyria, corpus callosum agenesis, hypopituitarism, hydrocephalus, microphthalmus), our patient was only noted to have macrocephaly and mild hypoplasia of the corpus callosum. No other brain structural abnormalities were present. Initial genetics evaluation with a microarray was normal, therefore further testing with exome sequencing is planned. We report an extremely rare case of bilateral ONA in an otherwise healthy infant and review the literature for the associated radiologic and genetic findings. Read More

Meeting name: IPR 2016 Conjoint Meeting & Exhibition , 2016

Authors: Lai Kuan-chi, Martinez-agosto Julian, Chawla Soni, Zipser Brian

Keywords: Optic nerve, aplasia, congenital

Orthotopic heart transplant (OHT) is the treatment of choice for patients with end-stage heart disease including pediatric patients. Imaging has an essential role in evaluation of the perioperative and postoperative heart transplant patients. Although some of the imaging is performed and interpreted by cardiologists, a substantial portion of images are read by radiologist, therefore radiologists must be familiar with common normal and abnormal posttreatment imaging features. Many end stage heart failure patients require circulatory support as a bridge to transplant, with a ventricular assist device or intra-aortic balloon pump. The ventricular assist devices specially increase the risk of infection, and intra-aortic balloon pump requires frequent radiographic monitoring to ensure appropriate placement in the proximal descending aorta, just distal to the left subclavian artery. Proximal placement may result in great vessel compromise and distal placement may cause occlusion of mesenteric and/or renal vasculature. Expected postoperative findings in the first few weeks after surgery included enlarged cardiac silhouette, small pneumomediastinum, pneumothorax, pneumopericardium, subcutaneous emphysema, small atelectasis, and mediastinal widening. Transplant-related complications are divide to: Early complications (0-30 days): pulmonary infection, interstitial and/or alveolar pulmonary edema, and allograft failure, also symptomatic pneumothorax, mediastinal hematoma, mediastinal infection, sternal dehiscence and vascular complications. Intermediate term complications (1-12 months): acute antibody related allograft rejection, acute cellular rejection (T-cell mediated response) which is the most common form of rejection, also tricuspid regurgitation, constrictive pericarditis, drug toxicity can happen in this time period. Late postoperative complications (>12 months): posttransplant lymphoproliferative disease, cardiac allograft vasculopathy, infection, kaposi sarcoma and other malignancies, aortic dissection, pseudoaneurysm formation, and thromboembolism. Read More

Meeting name: SPR 2020 Annual Meeting & Postgraduate Course , 2020

Authors: Saadat Vandad, Loo Jerry, Chen Johnathan, Iskander Paul, Chawla Soni, Ghahremani Shahnaz

Keywords: OHT, Complications, Imaging