Anand Neil,  Deochand Osmani,  Rios Jose

Final Pr. ID: Poster #: CR-001

The purpose of this exhibit is to make radiologists and clinicians aware of the radiological manifestations of Tularemia. There should be strong consideration for Tularemia in the differential in any patient with the classic symptoms, diffuse cervical lymphadenopathy with evidence of necrosis, and enlarged adenoids. Ultrasound and CT may demonstrate suppurative lymphadenopathy suggestive of infection; while chest radiography may demonstrate bilateral or lobar infiltrates. The radiological findings, prognosis, and clinical manifestations are to be discussed. Read More

Authors:  Anand Neil,  Deochand Osmani,  Rios Jose

Keywords:  Tularemia ,  ulceroglandular ,  Tularensis

Dewoolkar Suruchi,  Kazmi Faaiza,  Koenigsberg Robert

Final Pr. ID: Poster #: CR-002

The incidence of retropharyngeal abscesses in america is rising, despite the widespread use of antibiotics for symptoms of sore throat, fever, neck stiffness and stridor<!--EndFragment-->. Retropharyngeal abscesses were often considered a disease exclusively in children, requiring early recognition and aggressive management to prevent its significant risks of morbidity and mortality. The high rate of mortality associated with retropharyngeal abscesses is owed to its associated with airway obstruction, mediastinitis, aspiration pneumonia, epidural abscess, jugular venous thrombosis, necrotizing fasciitis, sepsis and erosion into the carotid artery. Often times, medical management and surgical incision and drainage attempts are unsuccessful and result in persistent abscess or worsened complications.

We studied two children, ages 9 months and age 11, each with a retropharyngeal abscess refractory to medical care. Both cases were in extremis, with respiratory and cardiac compromise. Read More

Authors:  Dewoolkar Suruchi,  Kazmi Faaiza,  Koenigsberg Robert

Keywords:  Percutaneous ,  Retropharyngeal ,  Ct-Guided

Shapira - Zaltsberg Gali,  Highmore Kerri

Final Pr. ID: Poster #: CR-003

We describe a case of a 14-year-old boy with a history of Legg-Calve- Perthes disease at the age of 6 and development of synovial osteochondromatosis (SOC) at the same hip joint 7 years later. Synovial osteochondromatosis is very rare in children and to the best of our knowledge, only a single case of Legg – Calve- Perthes disease and secondary synovial osteochondromatosis was described in the literature in a 35 year old male, making this the first reported case of Legg Calve Perthes disease and synovial osteochondromatosis in a pediatric patient.


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Authors:  Shapira - Zaltsberg Gali,  Highmore Kerri

Keywords:  Legg -Calve -Perthes ,  Synovial chondromatosis ,  Secondary synovial osteochondromatosis

Ledbetter Karyn,  Chernoguz Artur,  Shaaban Aimen,  Kraus Steven

Final Pr. ID: Poster #: CR-004

An 11-day-old male presented with recurrent choking episodes. Chest radiograph demonstrated partial opacification of the right hemithorax with associated air- bronchograms. Due to concern for tracheoesophageal fistula, a single-contrast barium esophagram was performed and demonstrated an anomalous bronchus arising from the distal esophagus. Subsequent CT of the chest showed an esophageal bronchus arising from the gastroesophageal junction with branches extending to the right lower and right upper lobes. No additional intrathoracic anomaly was identified. The patient was then treated with right upper lobectomy, right lower lobe apical segmentectomy and resection of the esophageal bronchus.

The esophageal bronchus is a rare communicating bronchopulmonary foregut malformation in which a bronchus arises from the esophagus. This anomalous bronchus may supply an entire lung or a single lobe. Although the lung parenchyma associated with the aberrant bronchus is classically supplied by the pulmonary circulation (thereby distinguishing it from a sequestration), additional anomalies are often seen. A few of the more frequently observed associated anomalies include pulmonary artery anomalies, esophageal atresia, duodenal atresia and tracheoesophageal fistula.

Diagnosing an esophageal bronchus begins with chest radiography, which will demonstrate a pattern of airspace disease that resembles a lobar pneumonia. This pattern of opacification is atypical in the neonatal setting, where the vast majority of pulmonary diseases, including pneumonia and aspiration, typically manifest as diffuse and bilateral processes. As patients with an esophageal bronchus also present with feeding difficulties, obtaining an esophagram is the next step in making the diagnosis. The esophagram will demonstrate filling of an abnormal structure that is often seen to be directed cranially and, with continued opacification, may be observed as a branching structure overlying opacified lung. Finally, cross-sectional imaging can be obtained to delineate the vasculature associated with the esophageal bronchus and to assess for additional anomalies.

The most common treatment of an esophageal bronchus is lobectomy. However, when the associated lung parenchyma is normal, performing an anastomosis between the esophageal bronchus and the tracheobronchial tree has been reported. On the contrary, pneumonectomy may be required in cases where anomalous vascularity precludes lobectomy. Read More

Authors:  Ledbetter Karyn,  Chernoguz Artur,  Shaaban Aimen,  Kraus Steven

Keywords:  Esophageal ,  Congenital

Albers Brittany,  Shah Chetan

Final Pr. ID: Poster #: CR-005

A previously healthy 12 year old female presented with left lower back pain and left thigh numbness. MRI showed a T1 isointense, heterogeneously T2 hyperintense left retroperitoneal mass with extension into the paraspinal muscles and intraspinal extension through the L1-L4 neural foramina. CT and contrast enhanced MRI (CE-MRI) of the lumbar spine and I-123 MIBG scintigraphy were performed. CT did not show any calcifications. MRI showed mildly heterogeneous, avid gadolinium enhancement. The mass was I-123 MIBG avid, without evidence of metastatic disease. Ultrasound guided biopsy yielded ganglion cells and no neuroblasts, suggestive of ganglioneuroma (GN). Partial excision of the retroperitoneal component yielded a 7 x 6 x 3 cm aggregate of tissue, and histopathology confirmed GN.

Follow up CE- MRI at 4, 10, and 16 months after surgery showed stable residual mass. CE-MRI at post op month 23 showed numerous T2 hyperintense enhancing osseous masses in the lumbar spine and sacrum. Residual mass remained stable. A fluoroscopically guided biopsy of a right sacral lesion yielded neuroblastoma. Review of the pathology from the original excision confirmed GN. Whole body I-123 MIBG scintigraphy showed the avid mass and confirmed skeletal metastases.

Ganglioneuromas (GN) are benign tumors of neural origin that exist on a spectrum with ganglioneuroblastoma (GNB) and the frankly malignant neuroblastoma (NB). CT, MR, and nuclear scintigraphy are unreliable for the differentiation of NB/GNB from GN. The most robust imaging feature to identify NB is the presence of distant metastases. Imaging findings that have been proposed as possible distinguishing features are the morphology of the calcifications, early versus delayed gadolinium enhancement, and ADC values. It is a well-known phenomenon that NB may regress into GNB or GN either spontaneously or following treatment with chemotherapy and/or radiation. To our knowledge, only one other case of GN de-differentiating into NB has been reported. Due to the extreme rarity of this case, alternatives must be considered. It is possible that the patient had a bi-phenotypic tumor and the NB component was not sampled initially or that she developed a NB extrinsic to the GN. These alternatives seem unlikely since NB is a rare tumor in 12 year olds, the residual tumor did not change on follow-up imaging, no new primary tumor was seen on recent MIBG, and two years passed prior to the development of metastasis. Read More

Authors:  Albers Brittany,  Shah Chetan

Keywords:  Ganglioneuroma ,  Neuroblastoma ,  Metastasis

Artunduaga Maddy,  Jadhav Siddharth,  Ketwaroo Pamela,  Masand Prakash

Final Pr. ID: Poster #: CR-006

Papillary fibroelastomas are benign primary cardiac tumors that usually arise from the valve apparatus and are rare in the pediatric population. Involvement of the tricuspid valve is even rarer, with only a few cases reported in the literature. We present the case of an 11-month-old female referred by her pediatrician after a non-cardiovascular acute visit to investigate a murmur noted since birth. The patient was asymptomatic. Family history was negative for congenital heart disease, seizures or genetic disorders. Her physical exam revealed a soft holosystolic murmur at the left lower sternal border but was otherwise normal. Initial work up with ECG and echocardiogram revealed limb lead reversal and a large mass in the right ventricle. Subsequent MRI showed a mobile 15mm oval mass along the septal surface of the right ventricle, in close proximity to the tricuspid valve, without valve inflow or right ventricular outflow tract obstruction. The mass was faintly visualized on TFE, isointense on T1, centrally-hyperintense and peripherally-hypointense on T2, and peripherally enhancing on post-contrast images. After a multidisciplinary review of the case, the decision was made to proceed with surgical resection. A preoperative CT of the chest did not reveal pulmonary embolism. During surgery, the mass was attached to the septal leaflet and the chordae of the tricuspid valve but not to the septum. Visually, the mass was compatible with a fibroelastoma. Off-bypass intraoperative transesophageal echocardiogram showed moderate tricuspid regurgitation that required on-bypass annuloplasty. Pathology confirmed the diagnosis of papillary fibroelastoma. There were no surgical complications. She was discharged on postoperative day 6 and had been doing well until her most recent follow-up four months after surgery. Papillary fibroelastomas are typically asymptomatic, although they carry potential risks for complications such as pulmonary or systemic emboli, depending on their location. MRI is a valuable examination that can characterize intracardiac tumors and thrombi. To our knowledge, this is the first report of a pathology-proven papillary fibroelastoma of the tricuspid valve characterized by MRI in such a young child Read More

Authors:  Artunduaga Maddy,  Jadhav Siddharth,  Ketwaroo Pamela,  Masand Prakash

Keywords:  Papillary fibroelastoma ,  MRI ,  intracardiac mass

Ledbetter Karyn,  Leino Daniel,  Trout Andrew

Final Pr. ID: Poster #: CR-007

Lipoblastomas are rare benign soft tissue tumors (6% of all pediatric soft tissue tumors) that are composed of immature lipoblasts and mature adipocytes. Lipoblastomas primarily occur in infants and children younger than three years of age and most frequently occur in the extremities. Less than 10 case reports exist of pelvic lipoblastomas. We report two children with pelvic lipoblastoma with the typical presentation of a rapidly enlarging, painless, fat-containing mass.

A 30-month-old girl presented with an enlarging gluteal mass over a period of four months. CT performed at an outside institution showed a large fat-containing intra-pelvic lesion anterior to the sacrum that extended to the perineum. An atypical sacrococcygeal teratoma was suspected. Subsequently, MRI demonstrated a large fairly-well-circumscribed intra-pelvic lesion that followed fat on all sequences with the exception of a few thin, enhancing septations.

A 14-year-old boy presented with a large firm scrotal/inguinal mass during a wellness visit. The child reported the lesion had been rapidly increasing in size over the past two months. Ultrasound showed a >15 cm inguinal mass that extended both into the pelvis and left hemiscrotum. The mass was homogeneous in echotexture and displaced but did not involve the epididymis and testicle. Based on concern for a malignant lesion, subsequent evaluation with PET-CT showed the lesion to be predominantly composed of fat with only low level F-18-FDG uptake in a few internal septations.

In both cases, lipoblastoma was confirmed after surgical excision with pathologic examination and immunohistochemistry.

Imaging serves to demonstrate the macroscopic fat content of lipoblastomas but cannot reliably differentiate lipoblastoma from liposarcoma. Therefore, imaging primarily contributes information about size and anatomic relationships relevant to surgical approach. Surgical excision is the treatment of choice; however, the lack of a capsule makes complete excision difficult and local recurrence is common. Close clinical follow-up is recommended for a minimum of five years, with imaging follow-up by MRI or CT utilized when the tumor’s anatomic location precludes or limits detection by physical examination. Read More

Authors:  Ledbetter Karyn,  Leino Daniel,  Trout Andrew

Keywords:  Lipoblastoma ,  Pelvic ,  Scrotal

Mizrahi Daniel,  Averill Lauren,  Blumer Steven,  Meyers Arthur

Final Pr. ID: Poster #: CR-008

This is a case report of a 15-year-old boy with chronic intermittent left shoulder pain due to an undiagnosed lesser tuberosity avulsion fracture, an associated biceps pulley injury and intra-articular dislocation of the long head of the biceps tendon. Also presented is a companion case of a 13-year-old boy with an acute lesser tuberosity avulsion with an intact biceps pulley. Avulsion fractures of the lesser tuberosity are rare injuries which can be difficult to diagnose clinically and on radiographs. Even more rare are reported cases of biceps pulley injuries in children and adolescents with lesser tuberosity avulsions. Utilizing the imaging from these two cases we review lesser tuberosity avulsions in children and also review the complex anatomy of the biceps pulley, which due to its close association with the lesser tuberosity, may be involved in these injuries. These cases emphasize the importance of MR in the diagnosis and treatment planning of lesser tuberosity avulsions. Read More

Authors:  Mizrahi Daniel,  Averill Lauren,  Blumer Steven,  Meyers Arthur

Keywords:  Lesser tuberosity avulsion ,  biceps tendon dislocation ,  biceps pulley injury

El-ali Alexander,  Vaughan Kevin,  Brown Amanda,  Davis Amy,  Grunwaldt Lorelei,  Sabri Yilmaz

Final Pr. ID: Poster #: CR-009

The aim of this report is to document the clinical presentation, multimodality imaging features, and management of patients with diffuse intra-abdominal Infantile Hemangioma (IH) and Kaposiform Hemangioendothelioma (KHE). Read More

Authors:  El-ali Alexander,  Vaughan Kevin,  Brown Amanda,  Davis Amy,  Grunwaldt Lorelei,  Sabri Yilmaz

Keywords:  Infantile Hemangioma ,  Kaposiform Hemangioendothelioma ,  Radiologic Pathologic Correlation

Dicamillo Paul,  Berlin Sheila,  Vasavada Pauravi

Final Pr. ID: Poster #: CR-010

Generalized arterial calcification of infancy (GACI) is a rare, often fatal disease due to cardiovascular sequellae (cyanosis, respiratory distress, hypertension and cardiomegaly) from widespread arterial calcification and/or narrowing of medium and large diameter vessels. Other findings can include periarticular calcification, pseudoxanthoma elasticum, hearing loss, intestinal ischemia, rickets and hypo/hyperphasphatemia. A database of worldwide cases implicates genes ENPP1 and ABCC6.

Our patient presented late in gestation. Although a 20 week fetal ultrasound was unremarkable, a 36 week ultrasound showed polyhydramnios, moderate pericardial effusion and moderate to severe tricuspid regurgitation; these findings prompted a C-section delivery. Early in his course, the patient developed biventricular dysfunction, systemic and pulmonary hypertension and respiratory failure requiring mechanical ventillation. Splenic calcifications, left pelvicaiectasis and lenticulostriate vasculopathy was documented in first week of life. The thoracic aorta, pulmonary artery and coronary artery were echogenic and thickened. Etidronate therapy, a treatment used for the first months to years of life to block bone mineralization until the arterial calcifications resolve, was started within 24 hours of life. However, this therapy can and did result in the development of rickets. Genetic testing revealed two mutations in the ABCC6 gene as can be seen in early onset GACI, a subtype with risk of pseudoxanthoma elasticum; our patient did exhibit hypermobile lower extremity joints. The patient's hypertension was eventually controlled with Amlodipine. Bulging fontenelles developed, likely due to ricket-impared skull growth. Calcification/narrowing of the bilateral carotids was seen. Additional complications included chronic pulmonary disease shown to be combination of chronic aspiration, nonspecific interstitial pneumonia and mild pulmonary arterial hypertensive changes. Rickets-related rib fractures further complicated the lung disease. Failure to thrive resulted in enteric feeding. Because of the severity of our patient's disease in which 6 month mortality can be as high as 85%, the treatment has aimed to prevent progression. Significant reduction in the arterial calcium burden has not yet been achieved, however the patient survived one year of treatment. Read More

Authors:  Dicamillo Paul,  Berlin Sheila,  Vasavada Pauravi

Keywords:  genetics ,  diphosphonate ,  hypertension

Fontalvo Herazo Lucia,  Putnins Rita,  Rotaru Carmen

Final Pr. ID: Poster #: CR-011

Idiopathic arterial calcification of infancy (IACI) is a rare condition, characterized by extensive depositions of hydroxyapatite in the internal elastic lamina of medium-sized and large arteries. It is usually diagnosed prenatally or in early infancy. Congenital intrahepatic portosystemic shunt is a persistent communication between the vitelline veins and sinus venosus, due to a focal absence of sinusoid formation. Infrequently they can present with signs of portal hypertension such as varices, ascites, and splenomegaly, but in most cases this feature usually indicates that the existing shunt is compensatory and not congenital.

We present a 29 week male neonate with a prenatal sonographic examination showing pericardial effusion, severe ascites and calcified cardiac outflow tract. After C section, the patient required resuscitation, intubation and abdominal drainage due to ascites in the NICU. Postnatal abdominal ultrasound confirmed abnormal calcification of the wall of the abdominal aorta, common iliac and origin of the superior mesenteric artery. Cranial ultrasound showed abnormal heterogeneous echotexture of the brain parenchyma with several linear parallel echogenicities, that may correspond to microcalcification of small caliber arteries. During the first few days of life the patient developed liver failure, severe cholestasis, coagulopathy and hypoalbuminemia. Follow up ultrasound done on day 12 showed hepatosplenomegaly, multiple hypoechoic focal liver lesions and an intrahepatic portosystemic shunt between the bifurcation of the main portal vein and the left hepatic vein. This finding was determined to be the primary cause of the ascites and progressive hepatosplenomegaly. By day 65 the portosystemic shunt had spontaneously resolved. Unfortunately the idiopathic arterial calcification had significantly progressed, despite bisphosphonate treatment. Multisystem organ failure had developed, the patient died on day 68.

DNA sequencing and high density target array analysis of ABCC6 and ENPP1 revealed no detectable mutation (anomaly most frequently related with IACI). Nevertheless, a c2320C>T transition in exon 23ENPP1 was identified. Finding been previously reported in a case of periarticular calcification.

This case illustrates two different and important pathologies that can be present in neonates. Each one has specific features and significant clinical relevance. This is a rare association as these two entities are usually seen separately. Read More

Authors:  Fontalvo Herazo Lucia,  Putnins Rita,  Rotaru Carmen

Keywords:  Idiopathic arterial calcification ,  Transient congenital porto systemic shunt ,  Peterme baby

Dance Logan,  Patel Mittun

Final Pr. ID: Poster #: CR-012

An otherwise healthy 37 day old male presented to dermatology with a pedunculated, friable red glabellar mass. First noticed as a flat, bluish lesion at 10 days, its subsequent rapid growth led to an emergency department visit where dermatology diagnosed a hemangioma and initiated propranolol treatment. Despite this, the mass continued to grow rapidly, encroaching upon the patient’s right eye. The patient was admitted for further workup. Ultrasound findings were consistent with hemangioma while MRI findings were atypical, contributing to a decision to obtain percutaneous biopsy. Pathology was consistent with choriocarcinoma. PET/CT found FDG-avid glabellar, liver and lung lesions.

The infant's therapy and clinical course will be briefly described. Clinical photos, ultrasound, MR, and PET/CT before and after therapy will be included.

Discussion

The unusual predilection of infantile malignancies for cutaneous metastases allows their early discovery and creates vivid clinical stigmata not easily forgotten. While relatively common in infancy, such presentations are exceedingly rare in children and adults.

In a series of 208 infants with cutaneous metastases, numerous bluish skin nodules created the so-called “blueberry muffin” baby appearance in 82% of cases. The following diseases presented with cutaneous involvement (ordered most to least common): leukemia, Langerhans cell histiocytosis, neuroblastoma, rhabdoid tumor, rhabdomyosarcoma, primitive neuroectodermal tumor, choriocarcinoma, and adrenocortical carcinoma.

Etiology and epidemiology will be discussed.

Imaging findings and a companion case in which choriocarcinoma was also misdiagnosed initially as infantile hemangioma will be discussed.

Considered one of the fastest growing tumors, infantile choriocarcinoma classically presents with hepatomegaly, anemia, failure to thrive, and precocious puberty between 0 days and 5 months of life. Left untreated, the disease is usually fatal within 3 weeks of presentation. Chemotherapy and delayed surgical excision of metastases can be curative with long-term survival rates of around 80%.

Conclusion

A solitary cutaneous metastasis can be mistaken for infantile hemangioma both clinically and radiographically. Atypical MRI appearance is one important clue that can suggest an alternative diagnosis. Early diagnosis and treatment of infantile choriocarcinoma is critical for survival. PET/CT may be useful for staging and follow-up. Read More

Authors:  Dance Logan,  Patel Mittun

Keywords:  choriocarcinoma ,  cutaneous metastases ,  infantile

Maller Vijetha,  Arora Anurag,  Boulden Thomas,  Choudhri Asim

Final Pr. ID: Poster #: CR-013

A newborn male child with antenatal diagnosis of left facial mass presumably hemangioma, presented for postnatal ultrasound. Ultrasound of the left face and neck, showed a large homogenous mass measuring 5x7x4 cm arising from the inferior aspect of the superficial lobe and entire deep lobe of the parotid gland extending over the angle of mandible to the left parapharyngeal space. On MRI the mass was heterogenously enhancing with low signal intensity on T1 and intermediate signal on T2 weighted imaging. The mass was also extending into the left masticator, sublingual, and submandibular spaces. The neonate also had a right upper abdominal swelling, and an ultrasound abdomen detected heterogeneously hypo to hyperechoic mass in the segment 4 of liver. On triple phase computed tomography, the mass showed early peripheral arterial enhancement and heterogeneous enhancement in portal and venous phase. On magnetic resonance imaging, the mass was hyperintense to liver on T2-weigthed imaging with restricted diffusion and heterogenous enhancement less than the background liver on all phases. Biopsy result of the left facial mass was sialoblastoma and that of liver was hepatoblastoma. Alpha fetoprotein was also elevated. Wide margin surgical resection of sialoblastoma and left lobe hepatectomy was performed followed by chemotherapy.

Sialoblastoma is a rare congenital tumor of the salivary glands, mainly arising from the parotid gland, which is locally aggressive and potentially malignant. A total of approximately 40 cases of congenital sialoblastoma has been reported. Neonatal hepatoblastomas comprises 10% of pediatric hepatoblastomas. An association of sialoblastoma and centromere division has been reported, which in turn is associated with embryonal tumors. There have been only two reported cases of concomitant sialoblastoma and hepatoblastoma. Further research with genetic analysis is required to document association of these two embryonal tumors. The current management of these tumors is complete surgical resection followed by chemotherapy. Read More

Authors:  Maller Vijetha,  Arora Anurag,  Boulden Thomas,  Choudhri Asim

Keywords:  Sialoblastoma ,  Hepatoblastoma

Oudjhane Kamaldine,  Alenezi Khaled,  Himidan Sharifa

Final Pr. ID: Poster #: CR-014

Congenital prepubic sinus is a rare congenital anomaly of the urinary tract and is clinically active by discharge from a skin opening overlying the symphysis pubis. Its etiology is still unclear, and a variant of dorsal duplication of the urethra is the favored theory. It has been reported in males as well as in females. Its size and extent are variable, starting from prepubic area extending toward the bladder or urethra and may have a blind end.. This report is about the imaging approach in a series of 5 cases with similar clinical signs of presentation.
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Authors:  Oudjhane Kamaldine,  Alenezi Khaled,  Himidan Sharifa

Keywords:  prepubic sinus ,  congenital ,  imaging

Mceniery Jane,  Corness Jonathan

Final Pr. ID: Poster #: CR-015

To report an unusual case of synchoronous primary brain tumours of two different histological subtypes in the posterior fossa of a 13 year old girl. Read More

Authors:  Mceniery Jane,  Corness Jonathan

Keywords:  Neuroradiology ,  Oncology ,  MRI