A 10-year-old female with neurofibromatosis type 1 and severe dysplastic scoliosis, presented with a two-month history of difficulty ambulating due to lower back pain. The patient did not have neurological symptoms. An MRI of the thoracolumbar spine demonstrated subluxation of the right tenth and eleventh ribs through the neural foramina, causing mass effect on the thecal sac, but at T9-T10 without abnormal cord signal or cord compression. Several clusters of neurofibromas were present along the right ribs and paravertebral soft tissues at these levels. CT evaluation was performed for greater osseous definition, which confirmed the findings. The patient underwent complex posterior spine fusion and segmental spinal instrumentation, which included T9-T10 thoracoplasty, rib resections, and multiple Ponte osteotomies. Cases of rib head protrusion into the central canal in the setting of dystrophic scoliosis have been documented in only a limited sample of case reports. The angulated short-segment curvature in dystrophic scoliosis causes vertebral body rotation, foraminal enlargement, spindling of transverse process, and penciling of the apical ribs. These changes can alter the articulation of the rib along the spinous process. The enlarged foramina also create a space into which a rib may displace. As a result, in most reported cases, the subluxed ribs were on the convex apex of the curve in the mid-to-lower thoracic region. The risk of cord injury makes complete depiction of the anatomy crucial for surgical management. Read More

Meeting name: IPR 2016 Conjoint Meeting & Exhibition , 2016

Authors: Chen Alan, Kucera Jennifer

Keywords: rib head protrusion, dystrophic scoliosis, spine

Neuroblastoma is the most common extracranial solid malignancy in children. It can have a variety of clinical outcomes, ranging from spontaneous resolution without therapy to fatal outcomes resistant to maximal therapy. Historically, neuroblastoma has been staged using the International Neuroblastoma Staging System (INSS). While this staging system has been used in clinical trials since its introduction in 1989, its reliance on surgical staging is problematic. Surgical resection can vary between surgeons and between tumors and occurs at an interval from diagnosis. This method complicates the process of standardizing therapy. Additionally, some patients have a disease that spontaneously regresses and does not require surgical management and thus cannot be staged. To combat these problems, the International Neuroblastoma Risk Group (INRG) created a new staging system for use in clinical trials in 2009. This staging system relies on preoperative imaging for up-front staging. This helps standardize neuroblastoma staging and helps to guide a more standard approach to management. The INRG staging system is comprised of twenty image-defined risk factors (IDRF), across multiple organ systems, which help predict surgical outcomes and can be combined with clinical data to provide up-front risk stratification. Even though the INRG staging system has been in use since 2009, many pediatric radiologists remain unfamiliar with its definitions and application. Additionally, MR has now become an essential imaging tool for diagnosis, staging, and follow-up of patients with neuroblastoma. The purpose of this poster is to compare the INSS and INRG staging system, describe the limitations of each system, and illustrate the definitions and IDRFs that comprise the INRG staging system. Read More

Meeting name: SPR 2018 Annual Meeting & Postgraduate Course , 2018

Authors: Chen Alan, Trout Andrew, Towbin Alexander

Keywords: neuroblastoma, IDRF, staging