Contenu connexe Similaire à NERRS Jan 2014 Pediatric Radiology Case Answers (20) NERRS Jan 2014 Pediatric Radiology Case Answers1. NERRS Jan 2014
Pediatric Radiology
Unknown Cases
Kirsten Ecklund, M.D.
Clinical Vice Chair of Radiology
Division Chief Musculoskeletal Imaging
Boston Children’s Hospital
Assistant Professor of Radiology
Harvard Medical School, Boston, MA
3. 7 y.o. boy with fatigue, knee pain and swelling
5. DDX Multifocal Marrow Abnormalities
• Neoplasm: Leukemia, metastatic dz
(NB, rhabdomyosarcoma)
• Infiltration: Gaucher’s, storage dz
• Reactive: severe anemias (red marrow)
• Infection/Inflammation: multifocal
osteomyelitis, CRMO
• Metabolic disease: Anorexia
nervosa, nutritional deficiency
6. Scurvy
• Vitamin C deficiency
– Vit C facilitates hydroxylation of lysine, proline
required for crosslinking collagen
– Deficiency impairs enchondral ossification
• Etiologies
– Nutritional deficiency
– Intestinal malabsorption
– Over utilization (thyrotoxicosis)
• Symptoms
– Lethargy, fever, malaise
– MSK sxs (80%) include bruising, joint pain
7. Scurvy
• Xray findings (late)
– Prominent ZPC (white lines of
Frankel)
– Subjacent lucent bands (scurvy
lines)
– Physeal widening
– Epiphyseal (Pelkan) spurs
– Metaphyseal fractures
• MR findings are non-specific
– Metaphyseal marrow changes
– Periosseous soft tissue edema
– Subperiosteal fluid/hematoma
(late)
• 3 very similar reported cases in
last 3 years
Skel Rad (2012) 41: 357-360
8. Scurvy
• Intramedullary and subperiosteal
hemorrhage
• Gelatinous transformation of marrow
• Our patient
– Marrow bx – fibrosis, hemorrhage
– Restricted diet: chicken
nuggets, water, packaged cookies
– Further eval: autism spectrum diagnosis
• 2 new cases in last 3 months
– Incidence of autism is increasing
10. 15 y.o. African American male with cough, shortness of breath, 10 # weight loss
13. Findings
• CXR – interstitial, septal
thickening
• Chest CT – septal
thickening, multiple small
nodules, ground glass opacities
• Abdominal CT –
infiltrative, indistinct left renal
mass
14. Pulmonary lymphangitic metastases
• Tumors: typically adenocarcinoma
– Breast, lung, colon, thyroid, pancreas
• CT Findings
– Interlobular septal thickening
– Nodules or reticulonodular changes
– Ground glass opacities
• DDX
– Sarcoidosis
– Idiopathic pulmonary fibrosis
– Pulmonary edema
15. Metastatic renal medullary carcinoma
• Demographics:
– Seen almost exclusively in pts with sickle trait
– Age range = 11 – 39, Male: female=3:1
• Symptoms: pain, hematuria, wt loss
• Renal mass imaging features
– Infiltrative, associated necrosis, caliectasis
– More commonly right-sided
• Often widely metastatic at dx
– Pulm lymph spread is common
17. 8 y.o. boy with abdominal
pain, distention and vomiting
18. Findings/ DDX
• SBO due to distal ileal stricture with
circumferential hyperemic soft tissue
• DDX
– Inflammation
• IBD
• Infection
• Focal inflammatory/post inflammatory process
– Neoplasm
• Lymphoma
• GIST
• Sarcoma
– Developmental
• Omphalomesenteric duct remnant
• volvulus
19. Inflammatory Myofibroblastic Tumor
• Synonyms: inflammatory pseudotumor, plasma cell
granuloma, pseudosarcoma
• Sites: Lung, orbit most common, but anywhere
• Etiology: ?, likely reactive
• Path: No malignant features, spindle
cells, mononuclear inflammatory cells
• Symptoms: depend upon site
– Abdomen: pain, fever most common
• RX: surgical resection, chemo?
• Prognosis
– Good for local, resectable dz
– Poor for infiltrative dz, esp abdominal, locally recurrent
20. IMT: imaging features
• Dependent upon anatomic site
• Typical: low density, +/- calc, focal mass
vs infiltrative soft tissue
• Bowel lesions: diffuse, infiltrative wall
thickening
• Overall, somewhat nonspecific
22. 11 y.o. boy with 6 months of bilateral knee pain, right > left
24. Findings
• Benign appearing tibial metaphyseal lesion
– Vascular (enhancement)
– No marrow edema or soft tissue mass
– Cortically based, sclerotic margins
• Systemic physeal widening
– Faint ZPC
– Metaphyseal fraying, osteopenia
25. Phosphaturic mesenchymal tumor
(PMT) with oncogenic osteomalacia
• Benign mesenchymal neoplasm
• Secretes phosphatonin (FGF 23)
– Inhibits renal phosphate reabsorption and conversion of
25-hydroxyvitamin D > 1,25 dihydroxyvitamin D
– Result is osteomalacia (rickets)- paraneoplastic
syndrome
• Tumor locations: extremities, facial bones
• Mesenchymal tumor types associated with OO:
– PMT, mixed connective tissue type
• Hemangiopericytoma
– Osteoblastoma-like tumors
– Ossifying fibroma-like tumors
– Non-ossifying fibroma-like tumors
26. PMT with osteomalacia
• Labs
– low serum phosphate, normal
calcium
– high 25-hydroxyvitamin D, low
1,25 dihydroxyvitamin D
– elevated FGF 23
• Imaging: Whole Body MR for
occult lesions
• RX: surgical resection (FGF
levels normalize in 1wk)
Notes de l'éditeur Aiden Moore 4091639 Xray findings reflect this impaired enchondral ossification in the setting of persistent resorption Davonte Neal 1274980 Davonte Neal 1274980 Davonte Neal 1274980 Abd CT done for pain, wt loss, transaminitis Atkinson 4600003 Berry 4590649Shevenell 4237902 (recurrent, required chemotherapy, multivisceraltxSpringer 4640349 Kidder 4509262MR: sag PD fs, cor PD, ax T1 fs post contrast FGF = fibroblast growth factor