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AJR 2007; 188:A196-A202
© American Roentgen Ray Society


ABSTRACT

Pediatrics

E439. Great Vessel Anomalies of the Heart in the Pediatric Patients. Assessment with Contrast-enhanced MR Angiography

Abdel Razek A.; Kandeel A.; Hafez M. Mansoura Faculty of Medicine, Mansoura, Egypt

Address correspondence to A. Abdel Razek (arazek{at}mans.eun.eg)

Background: Diagnosis of great vessels anomalies is limited with echocardiography and need to be examined with invasive angiocardiography. Contrast enhanced MR angiography is a promising noninvasive technique for diagnosis and preoperative mapping of great vessels anomalies of the heart in pediatric patients.

Key Issues: Seventy seven pediatric patients (52 M and 25 F, 3 months to 15 years old) with suspected congenital great vessels anomalies of the heart on echocardiography underwent contrast-enhanced MR angiography. Contrast enhanced MR angiography accurately diagnosed aortic coarctation (n = 19), aneurysm of the ascending aorta (n = 6), right sided or double aortic arch. It delineates pulmonary artery anomalies such as stenosis, hypoplasia or interruption in patients with tetralogy of Fallot and pulmonary atresia with VSD. Total (n = 4) or partial (n = 7) anomalous pulmonary venous drainage can be demonstrated. Bilateral superior vena cava (n = 3) and interrupted inferior vena cava (n = 5) can be clearly seen.

Format: The exhibit will be didactic in format presenting the types of great vessels anomalous of the heart followed by description of technique of contrast MR angiography of great vessels then description of characteristic MR angiography findings of aortic, pulmonary artery, pulmonary venous and systemic venous anomalous.

Teaching Points: To demonstrate the technique of contrast-enhanced MR angiography (CE-MRA) used for evaluation of congenital great vessel anomalies of the heart in pediatric patients. To review the spectrum of congenital vascular anomalies of the aorta, pulmonary artery, pulmonary veins and systemic veins detected with CE-MRA in pediatric patients.

E440. Great Vessel Anomalies of the Heart in the Pediatric Patient. Assessment with CT Angiography

Abdel Razek A.; Almrasafawey H. Mansoura Faculty of Medicine, Mansoura, Egypt

Address correspondence to A. Abdel Razek (arazek{at}mans.eun.eg)

Background: Diagnosis of great vessels anomalies is limited with echocardiography and need to be examined with invasive angiocardiography for decision of treatment. CT angiography with 3D reconstruction is a reliable noninvasive technique for diagnosis and preoperative mapping of pediatric congenital great vessels anomalies.

Key Issues: This study included 67 patients examined with CT angiography and 3D reconstruction. The findings were correlated with that of catheter angiography and surgery. Aortic arch anomalies were aortic coarctation, right sided aortic arch and double aortic arch. Pulmonary artery anomalies were either hypoplasia stenosis and interruption. Anomalous pulmonary venous drainage either total or partial was well demonstrated. Systemic venous anomalous were: persistent left superior vena cava and interrupted inferior vena cava. CT angiography showed patent ductus arteriosus, transposition of great arteries and double outlet right ventricle.

Format: The exhibit will be didactic in format presenting the types of great vessels anomalous of the heart followed by a description of techniques of CT angiography with 3D reconstruction of great vessels then a description of characteristic CT angiography findings of aortic, pulmonary artery, pulmonary venous and systemic venous anomalous.

Teaching Points: To describe technique of CT angiography (CTA) used for evaluation of congenital great vessel anomalies of the heart in pediatric patients. To review the spectrum of congenital vascular anomalies of the aorta, pulmonary artery, pulmonary veins and systemic veins detected with CTA in pediatric patients.

E441. Interrupted Aortic Arch Imaging

D'Amico A.; Yarram S. G.; Ensing G.; Hernandez R. University of Michigan, Ann Arbor, MI

Address correspondence to A. D'Amico (ardamico{at}med.umich.edu)

Background: Interrupted aortic arch (IAA) is defined as lack of luminal continuity between the ascending and descending aorta. It is classified into three types based on the location of the defect. Although IAA is a relatively rare condition, general knowledge of the classification and imaging appearance is important so that it can be recognized and accurately characterized to facilitate prompt and appropriate treatment given its high mortality rate. Untreated, IAA has a predictably high mortality rate of 90% at one year with a mean age of survival of only 4–10 days.

Key Issues: Surgical planning requires appropriate anatomic imaging to define the type of interruption, distance of separation, size of the ascending and descending aorta, branching pattern and location of the great vessels, status of the ductus arteriosus and left ventricular outflow tract, and identification of any other cardiac anomalies. Multiple imaging choices are currently available for IAA evaluation and appropriate workup depends on being familiar with the strengths and limitations of each. Although echocardiography is currently the primary modality, MRI has an increasing application in IAA and is most useful in complex cases due to excellent anatomic evaluation and ability to define coexisting cardiac anomalies.

Format: Didactic electronic presentation first defining IAA and discussing treatment. Various imaging modalities will then be explored with emphasis on MRI technique. Several classic and more complex case examples will also be presented which illustrate the findings of IAA.

Teaching Points: Explain the definition and characterization of IAA with background information on etiology and common associated diseases. Treatment and outcomes will be discussed with a focus on the role of imaging in surgical planning. Current imaging options will be presented with emphasis on MRI planning and interpretation including pitfalls that may lead to misdiagnosis as well as classic MRI appearances that aid in IAA characterization.

E442. Virtual and 3D CTA in the Evaluation of Variations in Patients with Bronchopulmonary Sequestrations

Richardson R. R.; Saghari H.; Stringfellow G.; Trahan A.; Goodman M. St. Joseph's Hospital and Medical Center, Phoenix, AZ

Address correspondence to R. Richardson (Randy.Richardson2{at}CHW.edu)

Objective: Bronchopulmonary foregut malformation can have a wide variety of manifestations. We present 16 cases of pulmonary sequestrations where CT angiography and MRI were used to make preoperative diagnosis and 3D reconstructions assisted surgeons in the operating room. A preoperative virtual bronchoscopy of a unique case of gastrobronchial fistula with anomalous arterial supply is also presented

Materials and Methods: 16 patients underwent CT angiograms of the chest with 3D rotational reconstruction and/or virtual endoscopy. We retrospectively reviewed 16 CT angiograms of the chest with 3D reconstruction in patients with pathology proven bronchopulmonary sequestration. CTA was performed using 3cc/kg nonionic contrast at 3 cc/sec and 2.5-mm axial slices.

Results: Of these 16 patients, 10 patients had a left lower lobe pulmonary sequestration (LLL) while 6 had a right lower lobe sequestration (RLL). All the RLL sequestrations had venous pulmonary drainage with one exception of one case of azygous venous drainage. The arterial supply for the RLL sequestrations was from the aorta in all the cases with exception of one case with aorta/celiac dual supply. A unique case of a gastrobronchial fistula is included in this series. Of the 10 LLL sequestration cases, 6 had arterial supply from the aorta, 2 had dual arterial supply, and one had an arterial supply from the descending artic arch. Other variations included: bilateral sequestrations, associated diaphragmatic hernia, left pleural effusion, left pneumothorax and an air filled cystic sequestration that was initially mistaken for a CCAM. 3D CT reconstructions were performed and were instrumental in guiding the surgeons in the operating room. In particular the gastrobronchial fistula case, a 3D bronchoscopy was also performed as a road map for the pulmonologist who performed the endoscopy but was unable to enter the anomalous gastrobronchial fistula.

Conclusion: There is a wide variety of presentations in patients with bronchopulmonary sequestration and CTA and MRA are uniquely equipped to preoperatively identify variations in arterial supply and venous drainage as well as the other associated anomalies.

E443. "Bubbles in the Chest": The Pediatric Spectrum of Air-containing Pulmonary Lesions

d'Almeida M.; Oneto J.; Lopez K.; Restrepo R. Miami Children's Hospital, Miami, FL

Address correspondence to M. d'Almeida (mdalmeid{at}msmc.com)

Background: The differential diagnosis of a partially or completely air-filled pulmonary lesion in children varies according to age, location and underlying conditions.

Key Issues: As the first step, radiologists should be familiar with the definition and imaging characteristics of all of the following air-containing lesions: bleb, bulla, cyst, pneumatocele, cavity, bronchocele and bronchiectasis. We will depict drawings and illustrate imaging characteristics of each disease with plain radiograph and computed tomography (CT).

Format: Grouping the diseases by categories is of major help in order to generate a differential diagnosis. A wide spectrum of diseases will be included such as necrotizing pneumonia, pulmonary abscesses, papillomatosis, eosinophilic granuloma, foreign body aspiration, kerosene inhalation, bronchial atresia, congenital developmental malformation of the lungs among others. Reference to diseases usually diagnosed in childhood associated with some of these conditions will be made including bullae and chronic lung disease of the premature, Bochdalek hernia and persistent pulmonary hypertension, subpleural blebs and connective tissue disorders, pulmonary abscesses and immunodeficiencies, bronchoceles and immotile cilia syndrome/cystic fibrosis, as well as pneumatoceles in the setting of pneumonia and trauma. Although special attention is given to diseases frequently and predominantly affecting children, some examples of adult diseases, less frequently affecting children will be shown, including: Wegener's granulomatosis, cavitary metastasis, septic emboli and alpha one antitrypsin deficiency among others.

Teaching Points: 1. To familiarize radiologists with the varied appearance of air-containing chest lesions in children. 2. To be able to generate a differential diagnosis of air-containing chest lesions based on the age of the patient, location and underlying conditions.

E444. Rhabdomyosarcoma: The Many Faces, Mimics, and Mimickers

Zell S. I.1,2; Brune B.1,2; Dalmeida M.1,2; Restrepo R.1,2 1. Miami Children's Hospital, Miami, FL; 2. Mount Sinai Medical Center, Miami Beach, FL

Address correspondence to S. Zell (szell21{at}gmail.com)

Background: Rhabdomyosarcoma, one of the blue cell tumors, is certainly more common in children than in adults. This tumor has a wide range of presentations, in part, due to a variety of places of origin, proving a challenge in its diagnosis. There are numerous atypical features of rhabdomyosarcoma which can mimic other pathologies, and add additional challenges to its radiological evaluation. Rhabdomyosarcoma may mimic benign entities such as cholesterol cyst, mastoiditis, muscle hematomas and ureteroceles, among others. On the contrary, various benign entities may simulate rhabdomyosarcoma. Some variations occur between the sexes, as well.

Key Issues: We are depicting cases arising from several origins: orbit, inner ear, nasopharynx, neck, biliary tree, peritoneum, urinary bladder, and striated muscles of the extremities. In girls, from the uterus/vagina, and in boys from the prostate and spermatic cord. Some of the tumors have presented originally with peritoneal carcinomatosis. A variety of cases will be shown depicting the multiple facets of this malignant tumor with typical and atypical presentations, as well as its mimickers using differential imaging modalities. Histologic correlation will also be presented.

Format: A didactic PowerPoint presentation including images, discussion and histologic correlation of rhabdomyosarcoma arising from multiple locations as discussed above, atypical features of rhabdomyosarcoma which can mimic other pathologies, and benign entities which can mimic rhabdomyosarcoma.

Teaching Points: 1. Understanding of and familiarity with the radiographic appearance of the typical and atypical locations and appearances of rhabdomyosarcoma. 2. Atypical features of rhabdomyosarcoma which can mimic other pathologies, including benign lesions. 3. Benign entities which can mimic rhabdomyosarcoma.

E445. Radiology of Lysosomal Storage Disorders

Kanekar S.; Pennypacker J.; Chetlen A. Penn State Milton Hershey Medical Center & College of Medicine, Hershey, PA

Address correspondence to S. Kanekar (san17kan{at}yahoo.com)

Background: Lysosomes are subcellular organelles responsible for the physiologic turnover of cell constituents, containing catabolic enzymes. Lysosomal storage disorders (LSD) is a group of genetic disorders that result from defective lysosomal metabolism resulting into cellular dysfunction. Due to nonspecific clinical findings various radiological imaging may provide a diagnosis or aid in limiting the differential diagnosis, which may in turn be diagnosed accurately by specific enzyme or substrate analysis. Till date, 61 lysosomal enzymes and 41 lysosomal enzyme disorders have been identified. We present the radiological manifestation of common and uncommon lysosomal disorders.

Key Issues: For the purpose of this exhibit and for easy understanding ofthese complex pathologies we have used the standard biochemical classification which depends on the enzyme deficiency and accumulation and / or excretion of toxic substrate. 1) Lipidosis: A) Sphingolipidosis-Fabry's, Gaucher's, GM1 and GM2 gangliosidosis, leukodystrophies like Krabbe's and metachromatic. Others-Niemann-Pick disease - Fabry's disease B) Nonsphingolipidosis Wolman's disease. 2) Mucopolysaccharoidosis. 3) Glycogenosis–Pompe's. 4) Glycoproteinoses–fucosidosis, mannosidosis. 5) Neuronal ceroid lipofuscinosis. 6) Mucolipidosis.

Format: Our presentation will be a didactic electronic exhibit. Participants will be able to navigate through a series of images and imaging features of various lysosomal storage disorders.

Teaching Points: We illustrate the characteristic imaging features of these various enzymatic defects. After the study of this exhibit the participant will be able to list and identify the radiographic findings in various LSD.

E446. Characterization of Diffuse Liver Disease in Pediatric Patients with MR Imaging

Abdel Razek A.; Abdalla A. Mansoura Faculty of Medicine, Mansoura, Egypt

Address correspondence to A. Abdel Razek (arazek{at}mans.eun.eg)

Background: Diffuse liver disease in pediatric patients is a common problem that may be due to cirrhosis, storage or metabolic disease or vascular causes.

Key Issues: Seventy-seven pediatric patients (48 boys and 29 girls aged 3 months to 15 years) with pathologically proven diffuse hepatic diseases underwent state of art MR imaging sequences. Regenerative nodules, lobar or segmental hepatic changes and collaterals were seen in cirrhotic patients. MR may help in characterization of storage disease as steatosis, iron overload disease, glycogen storage disease, Wilson's disease and Gaucher's disease. Enlarged caudate lobe with intrahepatic collaterals was detected in Budd Chiari syndrome. Other lesions such as cystic fibrosis and congenital hepatic fibrosis can be detected. We concluded that MR imaging can help in diagnosis and characterization of diffuse liver diseases in pediatric patients.

Format: The proposed exhibit will be didactic in format presenting MR pulse sequences used for examination of liver in pediatric patients by review of MR appearance of cirrhotic, metabolic, vascular, and other diffuse liver diseases in pediatric patients.

Teaching Points: 1) To review MR imaging sequences used for diagnosis of liver diseases in pediatric patients. 2) To demonstrate the MR features of cirrhosis liver diseases in pediatrics. 3) To review the MR appearance of metabolic and vascular liver disease in pediatrics.

E447. Multislice CT in Pediatric Small Bowel Lesions

Rajiah P.1; Maniyar A.2; Shabani A.2 1. Tameside General Hospital, Manchester, United Kingdom. 2. Manchester Childrens Hospital, Manchester, United Kingdom

Address correspondence to P. Rajiah (rprabhakar73{at}yahoo.com)

Background: Small bowel pathology in children ranges from inflammation to tumor and graft versus host disease. Multidetector CT is very useful in assessment of small bowel because contrast-enhanced slices enable assessment of bowel wall and also adjacent soft tissues. Knowledge of the specific imaging features helps in narrowing down differential diagnosis

Key Issues: The pictorial review starts with demonstration of the small bowel anatomy and physiology in children. The review illustrates the CT appearances of various pediatric small bowel lesions such as inflammatory bowel disease, tuberculosis, chronic granulomatous disease, cystic fibrosis, graft versus host disease, tumours, intussusception, malrotation and hernias. CT helps in assessing bowel wall and also adjacent soft tissues. Bowel wall thickening is a nonspecific finding, which should be assessed together with associated findings.

Format: PowerPoint/Flash presentation. Brief discussion of background, imaging features. Pictorial review illustrating the radiological findings in different imaging modalities. Differential diagnosis.

Teaching Points: 1. To discuss the optimal imaging protocol for assessment of small bowel pathologies in children. 2. To review the various pathological lesions affecting small bowel in children. 3. To understand the characteristic imaging appearance of small bowel lesions. 4. To illustrate the differential diagnosis of small bowel pathologies in children.

E448. Multidetector CT in Pediatric Abdominal Trauma

Rajiah P.1; Maniyar A.2; Shabani A.2 1. Tameside General Hospital, Manchester, United Kingdom. 2. Manchester Childrens Hospital, Manchester, United Kingdom

Address correspondence to P. Rajiah (rprabhakar73{at}yahoo.com)

Background: Imaging plays a vital role in assessment of pediatric trauma. Multidetector CT is very useful in assessing solid organ, visceral, vascular and bony injuries in abdomen and pelvis. CT is essential for grading trauma to solid organs such as liver, spleen, kidney, and pancreas, which is essential for determining appropriate management.

Key Issues: The pictorial review illustrates the various imaging findings in injuries to spleen (including shattered spleen and vascular injuries), kidney, liver, diaphragm, pancreas, bladder, bowel, mesentery, aorta, renal artery, vertebrae and pelvic bones. Shattered spleen, vascular injuries to spleen/kidney, extensive liver laceration, diaphragmatic rupture and bowel perforation require surgery.

Format: PowerPoint/Flash presentation. Brief discussion of background, imaging features. Pictorial review illustrating the radiological findings in different imaging modalities. Differential diagnosis.

Teaching Points: 1. To evaluate the role of multidetector CT in evaluation of pediatric abdominal trauma. 2. To review the various grading systems used in evaluating abdominal trauma in children. 3. To illustrate the CT appearances of various grades of abdominal trauma.

E449. Survey of Typical Radiographic Findings in `Survivors' with Stueve-Wiedemann Syndrome

Langer R. D.; Al Gazali L.; Raupp P. FMHS, Al Ain, United Arab Emirates

Address correspondence to R. Langer (rlanger{at}uaeu.ac.ae)

Objective: SWS is a rare skeletal dysplasia; most patients expire during infancy. Mutations responsible have been recently identified. The prevalence is low in the Western world, however, relatively high in the Gulf area (0.5/10,000 births in the UAE). Most SWS patients die before the age of two. To illustrate typical radiological findings in the small subgroup of patients with Stueve-Wiedemann Syndrome (SWS), surviving the 2nd year of age (`survivors')

Materials and Methods: Out of a total of 24 SWS patients, six survived the 2nd year. All children came to regular pediatric and orthopedic follow up examinations, or were admitted repeatedly due to respiratory distress. All patients received follow up bone and chest radiographs; one child with neurological deficit underwent cranial computed tomography (CCT). Radiological findings of our patient population are compared to few published data.

Results: All children showed congenital dwarfism with progressive bowing of the extremities, especially both legs, and generalized demineralization. Three patients had metaphyseal enlargements with coarse trabeculae. Three children developed progressive (kypho)-scoliosis, one spontaneous fracture of the femur and the contralateral fibula. Three children showed fragmented epiphyses. Epiphyseal flattening occurred in four patients. Metacarpals and metatarsals were shortened and undertubulated in all survivors. One patient developed an osteonecrosis of the talus, similar to radiological findings in insensitivity to pain. Rips were broadened and osteoporotic in all, showing old fractures in two cases. In all survivors also repeated chest radiographs were obtained, as a result of recurrent aspirations, and/or pulmonary infections. Two patients expired subsequently at the age of three and four years, respectively; both died from respiratory insufficiency. One girl developed a neurological deficit due to a left frontal infarction after an episode of hypoxia.

Conclusion: While Maroteaux still reported a mean survival of two months in 1995, several cases surviving infancy have been published since, and are at present under surveillance. SWS survivors after the 2nd year show progressive bowing of the (lower) extremities with generalized osteoporosis, a tendency to spontaneous fractures, metaphyseal abnormalities, and moderate to severe (kypho)-scoliosis. Pulmonary infections and aspirations are less frequently diagnosed after the 2nd year.

E450. The University of Miami Pediatric— Musculoskeletal Radiology Conference

Arraut A.; Shinault S.; Hancock C.; Parikh V.; Munoz R.; Segal G.; Clifford P. D. University of Miami MIller School of Medicine, Miami, FL

Address correspondence to A. Arraut (aarraut{at}um-jmh.org)

Background: This exhibit presents interesting and educational cases collected from the University of Miami's monthly pediatric-musculoskeletal radiology conference. A wide variety of entities that affect the pediatric musculoskeletal system are reviewed including bone and soft tissue tumors, trauma, sports injuries, infections, congenital anomalies and metabolic diseases.

Key Issues: The exhibit will discuss imaging findings of various pediatric musculoskeletal disease processes, with special attention paid to conventional radiographs and magnetic resonance imaging.

Format: The format is that of a series of case presentations. Relevant history and images are presented followed by a didactic description of the salient findings, the diagnosis and a brief up-to-date discussion of each entity as drawn from the literature, with commentary from faculty pediatric radiologists, pediatricians, orthopedists, oncologists and musculoskeletal radiologists.

Teaching Points: Teaching points of the exhibit include key diagnostic imaging findings, pertinent differential diagnoses and appropriate imaging workup of pediatric musculoskeletal cases.

E451. Variants of Exostosis of the Bone in Children

Richardson R.2; Delbridge C. J.1; Yellin J.1 1. Maricopa Integrated Health System, Phoenix, AZ; 2. St. Joseph's Hospital and Medical Center, Phoenix, AZ

Address correspondence to R. Richardson (randy.richardson2{at}chw.edu)

Background: Exostosis of the bone is a common benign radiographic finding in children. The appearance of a solitary exostosis of the bone can be characteristic and the diagnosis of an osteochondroma is often made without further imaging. Multiple osteochondromas are seen in multiple hereditary exostoses. Osteochondromas can be in specific locations such as the epiphysis in Trevors disease. There are also several osteochondroma-like lesions that can occur as normal variants or as the result of trauma, congenital anomalies, systemic disorders, or various causes of periosteal reaction.

Key Issues: Demonstrate and review common imaging characteristics of a benign osteochondroma. Demonstrate imaging features of hereditary conditions associated with exostoses. Demonstrate imaging findings of exostosislike lesions (i.e. normal variants, result of trauma, congenital anomalies, systemic disorders, or various causes of periosteal reaction.)

Format: The format will be an interactive computer exhibit demonstrating multiple images of an exostosis or exostosis-like lesions. Participants will have the opportunity to choose an answer of their proposed diagnosis. Following the participants answer choice, the correct answer will be given with radiologic pearls explaining the correct diagnosis and imaging findings.

Teaching Points: The major teaching points: 1. A review of benign osteochondromas. 2. Solitary exostosis of the bone have a characteristic appearance and can be diagnosed as an osteochondroma without further imaging modalities. 3. Know the hereditary conditions associated with exostoses. 4. There are multiple other causes of bony exostosis-like lesions that may be confused with an osteochondromas.

E452. Imaging Traumatic and Nontraumatic Spinal Lesions in Children

Rajiah P.1; Khan A.2 1. Tameside General Hospital, Manchester, United Kingdom. 2. SANG King Fahad Hospital, Riyadh, Saudi Arabia

Address correspondence to P. Rajiah (rprabhakar73{at}yahoo.com)

Background: There is a wide variety of spinal and cord lesions affecting children. Imaging plays an important role in diagnosis and localization of disease process.

Key Issues: The review illustrates the normal anatomy of spine and spinal cord in imaging modalities. The pictorial review illustrates the entire spectrum of spinal abnormalities, which includes, spinal malformations-scoliosis, segmentation anomalies, mucopolysaccharidoses, spine tumours-metastasis, lymphoma, Ewing's sarcoma, histiocytosis, spinal cord lesions-syringomyelia, tumours, meningioma, neurofibroma, epidermoid, metastasis, myelitis, demyelination, spinal cord malformations-dysraphism, vascular malformations. Differential diagnosis and imaging techniques are discussed.

Format: PowerPoint/Flash presentation. Brief discussion of background, imaging features. Pictorial review illustrating the radiological findings in different imaging modalities. Differential diagnosis.

Teaching Points: 1. To review the normal anatomy of the spine and spinal cord. 2. To review the common disease processes affecting the spine and spinal cord in children. 3. To discuss the optimal imaging protocols, in multislice CT and MRI.

E453. Pediatric Parotid Gland Disease

Goud A.1; Lowe L. H.2 1. Arrowhead Regional Medical Center, Department of Family Practice, Colton, CA; 2. Children's Mercy Hospital and Clinics and the University of Missouri-Kansas City, Kansas City, MO

Address correspondence to A. Goud (asha.goud{at}gmail.com)

Background: A variety of lesions occur in the pediatric parotid glands. With modern imaging techniques (Doppler US, helical CT and MRI), identification of a specific etiology is often possible. Clinical information, knowledge of normal anatomy and imaging characteristics of parotid space pathology are essential for appropriate radiological evaluation.

Format: The format of this exhibit will be a didactic, pictorial review of pediatric parotid gland imaging, including pertinent clinical history, embryology, anatomy and key radiographic features of various congenital, neoplastic, infectious and inflammatory processes.

Teaching Points: After viewing this scientific exhibit, the reader should: (1) Be familiar with the normal appearance of the parotid glands on various imaging studies. (2) Be able to formulate an imaging approach to pediatric parotid lesions using modalities such as ultrasound, helical CT, and MR imaging. (3) Be familiar with the imaging appearance of a variety of pediatric parotid lesions.

E454. Sonography of Focal and Diffuse Thyroid Lesions in Children and Adolescents

Masand P. M.; Siegel M. Mallinckrodt Institute of Radiology, St. Louis, MO

Address correspondence to P. Masand (masandp{at}mir.wustl.edu)

Objective: To characterize focal and diffuse thyroid lesions on sonography in the pediatric age group.

Materials and Methods: Sonograms and clinical data of 30 patients (3 years to 18 years, including 23 female and 7 male patients) were reviewed by two observers. All the patients had palpable thyroid enlargement. Sonographic assessment included gland size, echogenicity (hyper- or hypoechoic), homogeneity or heterogeneity, nodule formation, and vascularity. All the diagnoses were confirmed by biopsy or surgical excision.

Results: The thyroid masses were broadly classified into focal and diffuse lesions. Focal masses (n = 10) included adenomas (n = 6) and thyroglossal duct cysts (n = 4). Diffuse pathologies (n = 20) included Hashimoto's thyroiditis (n = 15), Graves disease (n = 3) and adenomatous hyperplasia (n = 2). Patients with adenomas were clinically euthyroid. Those with thyroglossal duct cysts presented as a midline neck mass. Patients with Hashimoto's thyroiditis were clinically hypothyroid, Graves' disease were hyperthyroid and those with adenomatous hyperplasia were euthyroid or hypothyroid. Focal adenomas on sonography were hypoechoic (n = 6) and associated with ipsilateral lobe enlargement (n = 6). Thyroglossal duct cysts were anechoic (n = 4) and occasionally septated (n = 2). Findings of Hashimoto's thyroiditis were diffuse heterogeneity with coarse parenchyma (n = 15), hypoechoic micronodules (n = 12) and increased vascularity (n = 15). Both patients with Graves' disease had homogeneous, diffusely hypoechoic and hypervascular glands and the three patients with adenomatous hyperplasia had heterogeneous glands with macronodule formation and hypovascularity.

Conclusion: Sonography is an invaluable tool in the assessment of focal and diffuse thyroid lesions in the pediatric population. Sonography can help differentiate focal from diffuse masses and also help differentiate among causes of diffuse and focal thyroid enlargement.

E455. Hemangioma and Vascular Malformations of Head and Neck: MR Imaging Characteristic

Abdel Razek A.; Saad E. Mansoura Faculty of Medicine, Mansoura, Egypt

Address correspondence to A. Abdel Razek (arazek{at}mans.eun.eg)

Background: There are different classifications of vascular malformation of the head and neck. MR imaging can diagnose and classify hemangiomas and vascular malformations of the head and neck that enable treatment targeted to the lesion.

Key Issues: Retrospective study on 82 patients with hemangiomas and vascular malformations of head and neck. Proliferating hemangiomas appeared as an intense enhancing mass that gradually decreased in size and involuating hemangiomas showed mixed signal intensity with fatty areas. Kapisoid hemangioendothelioma involves multiple spaces with prominent vessels. Signal void dysplastic veins with enhancement of slowing veins was seen in venous malformations and multiple dilated serpigenous signal void structures detected in arterial malformations. Lymphatic malformations commonly appeared as multiloculated multispatial cysts and mixed malformations contain different tissue components. We concluded that MR imaging can diagnose and classify hemangiomas and vascular malformations of the head and neck that enable treatment target to the lesion.

Format: The exhibit will be didactic in format presenting different classifications of hemangioma and vascular malformations of the head and neck followed by characteristic MR findings of each anomaly.

Teaching Points: 1) To understand classifications of hemangioma and vascular malformations of the head and neck. 2) To review the MR imaging characteristic of different stages of hemangioma 3) To demonstrate the MR imaging features of different types of vascular malformations.

E456. Neuroimaging of Tuberous Sclerosis: Spectrum of Pathology and Frontiers in Imaging

Kalantari B. N.; Salamon N. UCLA Medical Center, Los Angeles, CA

Address correspondence to B. Kalantari (bkalantari{at}mednet.ucla.edu)

Background: Tuberous sclerosis complex (TSC) is a multisystem congenital syndrome with widespread central nervous system anomalies. Clinical neurologic manifestations include epilepsy and cognitive impairments. Major intracranial features of TSC are cortical tubers, subependymal nodules, white matter lesions, and subependymal giant cell astrocytomas.

Key Issues: The number of tubers in the cortex and subcortex correlates with the clinical severity of disease. To identify the epileptogenic tuber from among many tubers is a challenge without invasive electroencephalography or intraoperative electrocorticography. Superimposition of functional information from 18F-FDG PET onto MRI has recently shown great promise in being able to accurately and noninvasively determine which tuber is epileptogenic, thereby improving surgical cure rates.

Format: The educational exhibit is didactic in format and organized by pathology, with an interactive quiz at the conclusion of the presentation.

Teaching Points: Following completion of the exhibit, one will be able to (1) identify the spectrum of CT and MR neuroimaging findings in TSC, and (2) employ PET/MRI fusion techniques to noninvasively localize epileptogenic tubers.

E457. Malformations of Cortical Development: MR Imaging Characteristic

Abdel Razek A.; Elmongy A.; Elserougy L. Mansoura Faculty of Medicine, Mansoura, Egypt

Address correspondence to A. Abdel Razek (arazek{at}mans.eun.eg)

Background: Malformations of cortical development vary according to stage of arrest during embryology. A wide variety of disorders of cortical development may be identified on MR imaging. Patients with malformations of cortical development may have a variety of abnormal brain findings, including abnormal gyral patterns, cortical thickening and white matter abnormalities.

Key Issues: Retrospective study of MR imaging for 55 pediatric patients with malformations of cortical development were done. MR imaging detects diminished cortical thickness and sulcation in microcephaly with abnormal cortex, enlarged and dysplastic cortex in hemimegalencephaly and focal thickening in focal cortical dysplasia. Malformative neoplastic disorders are ganglioglioma and dysembryoplastic neuroepithelial tumor. Smooth brain in classic lissencephaly differentiated from cobble stone cortex in congenital muscular dystrophy. MR detects types of grey matter heterotopia and schizencephaly and polymicrogyria. We concluded that MR imaging is essential to demonstrate the morphology, distribution and extent of malformations of cortical development.

Format: The exhibit will be didactic in format presenting embryologic stages of cortical development, classification of malformations of cortical development followed by key MR imaging findings of these malformations.

Teaching Points: 1) To review the embryological stages of cortical development. 2) To classify malformations of cortical formation according to embryological stages. 3) To demonstrate the MR appearance of malformation of cortical development.

E458. Role of Diffusion Weighted MR Imaging and Proton MR Spectroscopy of the Brain in Pediatric Patients with Chronic Liver Disease

Abdel Razek A.; Abdalla A.; Galal A. Mansoura Faculty of Medicine, Mansoura, Egypt

Address correspondence to A. Abdel Razek (arazek{at}mans.eun.eg)

Objective: To determine the role of diffusion weighted MR imaging and proton MR spectroscopy of the brain in pediatric patients with chronic liver disease.

Materials and Methods: Diffusion-weighted MR imaging (DWI) and multivoxel proton MR spectroscopy of the brain were done for 37 pediatric patients (22M, 15F aged 4 to 14 years: mean 9 years) with chronic liver disease and 20 volunteers as control. Diffusion MR weighted imaging was done using a single shot echo planar imaging with a diffusion-weighted factor b of 0, 500 and1000 sec/mm2. The apparent diffusion coefficient (ADC) map was reconstructed. Proton MR spectroscopy (multivoxel PRESS technique: TR/TE = 1500/135, 1.6 x 1.6 matrix, FOV = 100 mm, 2 averages) was performed at the level of most significant MR abnormality. Assignment of the resonance peaks of NAA, creatine, choline, glutamate, myinositol, lipid and lactate were determined in the basal ganglion, thalamus, grey and white matter.

Results: Restricted diffusion (high SI at high diffusion-weighted images and low SI at ADC maps) within the corticospinal tract in hepatic encephalopathy (n = 14), putamen and caudate nucleus in Wilson disease (n = 4) and frontal and occipital white matter in Gaucher's disease (n = 2). MR spectra revealed increased glutamate with decreased myoinositol and choline peaks in hepatic encephalopathy. Wilson disease showed decreased choline and myoinositol that associated with lactate. MR spectra in Gaucher's disease revealed presence of lipid and lactate.

Conclusion: Diffusion-weighted MR imaging and proton MR spectroscopy added valuable information to routine MR imaging of the brain in pediatric patients with chronic liver disease.

E459. What is Additive Value of Transcranial Doppler to Conventional Neonatal Gray Scale Head Ultrasound?

Barak R.2; Fadell M. F.2 1. Columbus Children's Hospital, Columbus, OH; 2. Medical University of Ohio, Toledo, OH

Address correspondence to R. Barak (rakeshbarak{at}gmail.com)

Background: In addition to the information provided by transcranial grey scale sonographic examination of the pediatric brain, transcranial Doppler imaging can provide valuable diagnostic information in a number of neonatal conditions.

Key Issues: The exhibit will discuss the technical factors required to successfully perform transcranial Doppler in the neonate, as well as review the normal neonatal transcranial vascular anatomy and Doppler waveforms. Alterations in color and pulsed Doppler findings in various pathologic conditions and their associated physiologic changes will also be discussed.

Format: This didactic exhibit will review normal anatomy, as well as discuss indications and techniques for neonatal transcranial Doppler ultrasound. Imaging examples with pathophysiologic correlation will address the following: intracranial hemorrhage, hypoxia/ischemia, hydrocephalus, left to right shunts, vascular malformations, extra-axial fluid collections, venous thrombosis, and brain death. Limitations and advancements in transcranial Doppler ultrasound will also be discussed.

Teaching Points: 1. The diagnostic benefits of using alternate acoustic windows in transcranial Doppler evaluation of the neonatal brain. 2. How neonatal transcranial Doppler imaging can provide valuable diagnostic information in addition to standard neurosonography in certain neonatal conditions. 3. How neonatal transcranial Doppler imaging can make neonatal neurosonography more than just a screening modality.

E460. Continually Enlarging Comprehensive World Wide Web-based Pediatric Radiology Teaching File, Utilized as a Model to Increase Exposure to New Technologies.

Zakhary B.1; Silberberg P.1; Schmitz J.1; Reyna R.2; Aremu I.4; Jimoh K.4; Gillespie V.5; Lyda L.3 1. Creighton University and Omaha Children's Hospital, Omaha, NE; 2. Hospital Santo Tomas, Panama City, Panama; 3. The Angkor Hospital for Children, Siem Reap, Cambodia; 4. University of Ilorin Teaching Hospital, Kwara State, Nigeria; 5. www.humaninet.org, Portland, OR

Address correspondence to B. Zakhary (bzakhary{at}creighton.edu)

Background: www.mypacs.net is an online repository of radiology cases used as a source for reference and teaching. With more than 13,000 cases and 50,000 images, myPACS.net has become the largest teaching file collection on the Web. Utilizing the curriculum developed by the Society for Pediatric Radiology, we present a Web-based collection of pediatric radiological cases by anatomic and disease categories. This will complement existing resources and will attempt to increase exposure to tropical diseases seen in pediatric patients. To augment the database, we have added cases from our own departments, personal collections, and third-party sources shared by colleagues in Africa, Asia, and Central and South America.

Key Issues: Our collection is a nonproprietary computer-based approach enabling sharing of images and diagnostic data. Internet capabilities allow a collaborative effort for the collection and sharing of these images. This is valuable to those learning and also practicing as a knowledge resource at the point of care. New technologies will be highlighted. These include (a) the creation of hierarchies utilizing dynamic anatomic and pathologic hierarchies which can easily be created and modified and (b) recently available technology which allows Internet connectivity in places in the developing world where this has previously been unobtainable (see www.humaninet.org).

Format: The exhibit will be interactive and organized by anatomic and pathologic process and informational with respect to the two new technologies.

Teaching Points: 1. Increase exposure to a continually enlarging comprehensive collection of pediatric radiology Web-based teaching files, while enhancing the learning potential for medical students, residents, fellows, caregivers, and practicing physicians. 2. Increase the collection of pediatric tropical radiology learning resources on the World Wide Web. 3. Highlight a recently developed technology where dynamic anatomic and pathologic hierarchies can be developed for all different radiological subspecialties and throughout medicine. 4. Increase exposure to and enlighten people in the developing world to novel ways to obtain Internet connectivity utilizing recently released technology.

E461. A PDA Reference Database Solution for Pediatric Plain Film Radiography

Talanow R.1; Paetzel M.3; Grunewald M.2 1. Cleveland Clinic Foundation, Cleveland, OH; 2. Clinic of Nuclear Medicine, University of Erlangen-Nuremberg, Erlangen, Germany; 3. EduRad, Cleveland Heights, OH

Address correspondence to R. Talanow (arrs.org{at}talanow.info)

Background: It is especially difficult for the inexperienced radiologist or the resident on-call to differentiate between physiological age-specific and pathological findings due to developmental variations in the growing child. The suitable book is often unavailable. To solve this problem an easy accessible format like handhelds would help in this situation.

Key Issues: We developed a free program for handheld devices as a reference database in pediatric plain film radiography which helps the well- and less-experienced pediatric radiologist differentiate between physiological age-specific and pathological findings. The comprehensive but user friendly structure of this program allows the user to quickly find the needed images for comparison.

Format: More than 450 images in pediatric skeletal radiography can be displayed in radiographic standard settings on a handheld device. All images are categorized by body regions, age, and gender. The database can be queried through selection of all three criteria or by choosing each criterion separately. The latter offers itself mostly for determination of bone age using the left hand. Each mode provides the user with at least one reference image according to each body region, age and gender.

Teaching Points: This program is a free reference database for almost all standard images in plain film radiography, from infancy to adulthood. The database serves the radiologist on-call and the less experienced pediatric radiologist to differentiate between physiological age-specific and pathological findings.

E462. An Online Reference Database in Pediatric Plain Film Radiography

Talanow R.1; Grunewald M.2; Wagner M.2; Greess H.2; Bautz W.3; Uhl M.4 1. Cleveland Clinic Foundation, Cleveland, OH; 2. Clinic of Nuclear Medicine, University of Erlangen-Nuremberg, Erlangen, Germany; 3. University Hospitals of Erlangen, Erlangen, Germany; 4. University Hospitals of Freiburg, Freiburg, Germany

Address correspondence to R. Talanow (arrs.org{at}talanow.info)

Background: In pediatric skeletal imaging it is difficult especially for the inexperienced radiologist or the resident on-call to decide what is a physiological variation and what should be deemed as pathological due to age-specific variations of the child's bone. The suitable book is often unavailable. To solve this problem an easy accessible format like a Web-based reference database would help in this situation.

Key Issues: We developed a free Web-based program as a reference database in pediatric plain film radiography which helps the well- and less-experienced pediatric radiologist differentiate between physiological age-specific and pathological findings. The comprehensive but user friendly structure of this program allows the user to quickly find the needed images for comparison.

Format: More than 450 images in pediatric skeletal radiography can be displayed in radiographic standard settings on a provided Website. All images are categorized by body regions, age and gender. The database can be queried through selection of all three criteria or by choosing each criterion separately. The latter offers itself mostly for determination of bone age using the left hand. Each mode provides the user with at least one reference image according to each body region, age and gender.

Teaching Points: This program is a reference database for almost all standard images in plain film radiography, from infancy to adulthood. It is freely accessible over the Internet under http://www.tnt-radiology.de/pedbone/. The database helps the radiologist on-call and the less experienced pediatric radiologist differentiate between physiological age-specific and pathological findings.

E463. Imaging of Gaucher Disease: Common and Atypical Findings

Green B.2; Lowe L. H.1; McDonald M. E.1 1. Children's Mercy Hospital and Clinics and the University of Missouri-Kansas City, Kansas City, MO; 2. University of Missouri, Columbia, MO

Address correspondence to B. Green (greenbr{at}health.missouri.edu)

Background: Gaucher disease, though rare, is the most common lipid storage disorder. Because of its effect on the reticuloendothelial system, multiple organ systems are involved, leading to a wide array of imaging findings. Since the advent of enzyme therapy to treat Gaucher's disease, imaging studies play a critical role in staging disease and monitoring a response to treatment. It is, therefore, becoming increasingly important for radiologists to be familiar with the multisystem imaging findings associated with Gaucher's disease.

Key Issues: This exhibit will discuss and illustrate the imaging findings of Gaucher's disease expected before and after enzyme therapy. Key radiological features of the gastrointestinal, musculoskeletal and pulmonary systems will be emphasized with various imaging techniques including plain radiography, sonography, CT and MR imaging. A description of the various methods currently used to monitor treatment of Gaucher's disease will also be covered.

Format: The format of this exhibit will be a didactic, pictorial review of the characteristic and atypical imaging features of Gaucher's disease.

Teaching Points: After reviewing this exhibit, the reader will: (1) be familiar with typical imaging findings of Gaucher Disease in all pertinent organ systems, (2) be aware of how to interpret and the impact of imaging on treatment and staging of disease and (3) understand specific imaging methods used to assess response to treatment.


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