Ileocolic intussusception is a common diagnosis routinely treated by air or contrast enema in the pediatric setting. Cases become less common into the second decade of life with an inverse relationship with pathologic lead points as the inciting culprit. Idiopathic ileocolic intussusception in young pediatric patients has been correlated with seasonality and viral infections, leading to the hypothesis that lymphoid hypertrophy in the terminal ileum acts as the primary lead point in that patient population. Pediatric radiologists are vigilant in evaluation of underlying pathologic lead points in the older population including mechanical causes (Meckel diverticula, polyps, duplication cysts), lymphoma, or hematomas from bleeding disorders (IgA vasculitis, hereditary coagulopathies). Adult surgeons often operate on patients with ileocolic intussusception given the much higher propensity for pathologic lead points. A 20-year-old patient presented to the emergency room with acute onset severe colicky abdominal pain that awoke her from sleep. CT of the abdomen and pelvis was performed showing a right lower quadrant ileocolic intussusception. Patient was otherwise stable and referred to pediatric radiology for successful air enema reduction and planned for further outpatient work up of possible lead points. The patient experienced recurrence of symptoms 1 day later and was taken to the operating room where recurrent ileocolic intussusception was confirmed and ileocecetomy was performed. Pathologic examination of the specimen obtained during surgery revealed reactive lymphoid hyperplasia and multiple reactive mesenteric lymph nodes. Read More

Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024

Authors: Burns Madisen, Cao Joseph

Keywords: Intussusception

Major aortopulmonary collateral arteries (MAPCAs) are congenital vessels derived from the splanchnic vascular plexus connecting the systemic circulation to the pulmonary arterial vasculature. These abnormal collateral vessels persist after birth when there is disruption of normal antegrade flow through the pulmonary arteries. MAPCAs are most commonly seen in patients with Tetralogy of Fallot with pulmonary atresia (TOF/PA). Our patient was prenatally diagnosed with hypoplastic left heart syndrome (HLH) and anomalous pulmonary venous return. She was born at term with an initial CT angiogram showing obstructive supracardiac total anomalous pulmonary venous return (TAPVR) and a small sub-millimeter MAPCA. The patient underwent immediate vertical vein stenting and subsequently underwent Norwood and open TAPVR repair on day of life 16. She continued to clinically deteriorate due to elevated pulmonary arterial pressures and was eventually diagnosed with occlusion of her left pulmonary veins, confirmed on repeat CT angiography where the small MAPCA had increased to over 2 mm in size. The patient continued to experience pulmonary hypertension resulting in respiratory failure requiring intubation and eventual extracorporeal membranous oxygenation. She became hypoxic and bradycardic during operative tracheostomy placement and was unable to be resuscitated. Read More

Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024

Authors: Burns Madisen, Cao Joseph

Keywords: Pulmonary, Congenital, Cardiac

This exhibit reviews the major congenital heart lesions affecting the right ventricular outflow tract (RVOT) and the surgical techniques performed in correcting these lesions. We explore the immediate post-surgical appearance of these cases through ultrasound echocardiography, CT angiography, and cardiac MR angiography. The final section reviews the more common complications seen with RVOT intervention. Read More

Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024

Authors: Burns Madisen, Cao Joseph

Keywords: Congenital, RVOT, Cardiac

Background: Granular cell tumors (GCT) are rare Schwann cell-derived soft tissue tumors typically presenting as a solitary, painless nodule in adults in the 4th to 6th decades of life, rarely presenting in children. GCT most commonly originate from gastrointestinal mucosa of the tongue or oral cavity, and are 2-3 times more common in women. While generally benign, approximately 2% of cases may be malignant; therefore, GCTs are typically treated with local surgical control. Case: A 7-year-old male with sickle cell trait presented to clinic for a painless “pea-sized lump” on his penile shaft, which had been present since infancy but had recently grown larger. Ultrasound demonstrated a 1.8 cm lenticular-shaped mass on the dorsal mid shaft of the penis superficial to the fascial borders of the cavernous cavernosum. There was mild internal vascularity, but no invasion past fascial borders, surrounding soft tissue changes. On MRI, the mass was uniformly T1 iso-intense and T2 slightly hyperintense, with slight postcontrast enhancement. The mass was well-circumscribed, wrapped around the corpus cavernosum without invasion of the tunica albuginea, and exhibited uniform restricted diffusion, calcification, or internal fat. No lymph node involvement was noted on imaging. Surgical resection 8 months later noted a 3 cm mass, which now extended into the glans, with pathology consistent with benign GCT, with positive staining for S-100, CD68, SOX10, and calretinin, Ki-67 of 1%, and negative for SM actin. Due to depth of glans penetration, R2 resection was achieved based on the pediatric urologist’s decision to spare the glans, rather than performing a more aggressive distal penectomy. Post-operative MRI 4 months later demonstrated residual tumor with interval reduction in mass size. Additional investigation of the patient’s past medical history revealed that a benign GCT had been removed from the inside of his lip the previous year which helped support the final histopathologic diagnosis. While multifocal GCTs have been described in genetic syndromes, this patient had not undergone genetic testing to date. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Beckhorn Catherine, Lee Rachel, Cao Joseph

Keywords: MRI, Genitourinary, Soft-Tissue Tumors

Keratin-positive giant cell-rich tumor is a very rare subset of giant cell-rich tumors characterized by keratin-positive cells and HMGA2::NCOR2 gene fusion. First described in 2021, fewer than 40 cases have been reported in the English literature. Reported cases have shown a strong predilection for females in their 20s-30s and occur in both soft tissue and bone. The youngest reported case thus far has been a 13-year-old female. A 4-year-old previously healthy male presented to clinic with leg pain and a limp after falling on his right leg. Initial X-ray showed a lytic lesion on the right proximal tibia. Follow up MRI showed a 2 x 2.5 x 4.3 cm solid lesion in the proximal tibial metaphysis extending into the epiphysis. The mass was T1 and T2 hyperintense and demonstrated homogenous enhancement after contrast. No periostitis was noted on radiograph and no subperiosteal collection was present. No soft tissue component was identified. CT chest, abdomen, pelvis was ordered to rule out other sites of disease and showed multi-station lymphadenopathy throughout the right groin. Biopsy revealed tumor cells negative for H3G34W and H3K36M histone markers, specific for giant cell tumor of bone and chondroblastoma respectively. The cells were focally positive for AE1/AE3, a stain for keratin. Next-generation sequencing revealed an HMGA2::NCOR2 fusion confirming the diagnosis of keratin-positive giant cell-rich tumor. He underwent complete curettage of the lesion as well as excision of 2 inguinal lymph nodes that were negative for disease. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Lee Rachel, Beckhorn Catherine, Cao Joseph

Keywords: Bone Tumor, Musculoskeletal

Aortic calcifications are an uncommon finding in the pediatric population and when present are often reflective of underlying congenital heart or metabolic disease. Intraluminal polypoid calcified lesions (coral reef aorta) causing flow limiting stenosis are exceedingly rare in even the congenital heart disease population. A 15-year-old patient with a history of hypoplastic left heart syndrome (HLHS) with mitral and aortic atresia. She was found to have significant flow limiting stenosis of the augmented neo-aorta under catheter hemodynamic assessment. CT angiography was performed showing a 4.4 cm partially calcified mass in the ascending aorta extending into the proximal arch causing an 80% narrowing of the aortic lumen. The intrinsic multi-energy capabilities of the first-generation photon counting CT (PCCT) scanner used permitted retrospective material decomposition and thus more definitive characterization of the mass. The patient underwent open ascending aorta replacement and arch repair where densely calcified ascending aorta and base of the arch were removed en bloc along with a large intraluminal mass component without complication. Pathologic assessment revealed PTFE conduit material, thrombus, and extensive calcification with fibrous incorporation of graft and arterial wall. Read More

Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024

Authors: Burns Madisen, Cao Joseph, Carrico Caroline

Keywords: Aorta, Calcifications, Stenosis

Photon-counting detector (PCD) CT utilizes a charged semiconductor element directly paired with a high-resolution detector panel. The direct conversion of detected photons into electrical signal represents the most recent generational advance in CT technology. Changes to detector technology achieved with PCDs has the potential to reduce image noise, improve spatial resolution, improve contrast resolution, and provide multispectral imaging capability. Importantly, these gains may be achieved with an overall decrease in radiation and iodinated contrast dose. This exhibit reviews the underlying technology of the PCD using clinical scans of pediatric patients performed on the first commercially available PCCT. We present examples of (1) PCD noise reduction capability; (2) improvements in spatial resolution; (3) improvements in contrast resolution; and (4) uses of multispectral capability. These are presented in comparison to the latest generation energy integrating detector (EID) CTs to highlight reductions in radiation dose and contrast media dose. Read More

Meeting name: SPR 2023 Annual Meeting & Postgraduate Course , 2023

Authors: Cao Joseph, El-ali Alexander, Siegel Marilyn

Keywords: photon counting, CT, ALARA