Ibe Donald,  Navallas Irujo Maria,  Aquino Michael

Final Pr. ID: Poster #: CR-001

Lemierre’s syndrome is an extremely rare condition characterized by initial oropharyngeal infection with development of septic thrombophlebitis and subsequently disseminated septic microemboli. The syndrome remains a disease of considerable morbidity and mortality. The incidence is approximately 3.6 cases per 1 million per year. It is commonly caused by gram-negative Fusobacterium necrophorum. However, less than a third of cases is brought on by other anaerobic bacteria. Here we present a rare case of a Lemierre’s syndrome in a child caused by methicillin-resistant Staphylococcus aureus (MRSA).

A 4-year old male presented to emergency department with unremitting fever, progressive painful submandibular swelling concerning for Ludwig’s angina, vesicular lesions on the skin, and decreased level of consciousness. The patient was reported to have fallen onto his chin with resultant lip laceration and tooth avulsion. Fever and neck swelling developed two days later. Computed tomography (CT) of the neck revealed findings in keeping with clinically suspected Ludwig’s angina including: soft tissue gas, and diffuse fat stranding involving the sublingual, perioral, and right submandibular spaces with extension to right sternoclavicular muscle, carotid and jugular vessels. No abscess was identified but a focal non-occlusive thrombus was seen in the right internal jugular vein. Additionally, the lung apices demonstrated multiple patchy densities raising concern for Lemierre’s syndrome and prompting further evaluation with a contrast-enhanced chest CT. Chest CT confirmed the diagnosis demonstrating multiple, variable-sized, randomly distributed lung nodules with cavitation, and multifocal consolidation consistent with septic emboli. Blood culture and skin swab of vesicular lesions were positive for MRSA. On further discussion, it was revealed that the patient’s father was recently treated for MRSA abscess. The patient was placed on intravenous antibiotics (vancomycin, rifampin, meropenem) and anticoagulants (tinazaparin) with improvements in symptoms and imaging findings within six weeks post admission.

The learning points include:
1) the need to critically evaluate lung apices and vasculature on neck CT in patients with evidence of soft tissue neck/oropharyngeal infection
2) despite the rarity of Lemierre’s syndrome, multiple cases caused by MRSA have been described. Read More

Authors:  Ibe Donald,  Navallas Irujo Maria,  Aquino Michael

Keywords:  SEPTIC EMBOLI ,  THROMBOPHLEBITIS ,  ludwig's angina

Evens Ashley,  Gonzalez-gomez Ignacio,  Neville Kucera Jennifer

Final Pr. ID: Poster #: CR-002

Infantile myofibromatosis is a rare condition consisting of benign fibrous tumors typically deposited in the skin, soft tissues, muscles, bones, and visceral organs. The entity can be solitary or multicentric. Although controversial, outcomes are generally worse in cases with visceral organ involvement. The prognosis is generally favorable in cases that lack visceral organ involvement, with a majority of cases showing spontaneous regression.

The imaging findings of infantile myofibromatosis will be illustrated using both prenatal and postnatal imaging including ultrasound, MRI, radiography, CT, and bone scintigraphy. We also present gross specimen and pathology images.

Our case involves a 33 week 4 day gestational age male fetus that initially revealed dilated loops of bowel on ultrasound. Fetal MRI was performed at 34 weeks and 4 days, which demonstrated the dilated loop was colon in the region of the hepatic flexure. Additionally, multiple solid-appearing lung masses were noted, which had not been visualized on ultrasound. Because of concern for a possible metastatic process, the entire fetus was thoroughly imaged, but no primary source was found. The differential diagnosis that was given on the fetal MRI included metastatic disease from the mother or fetus, infantile myofibromatosis, or infectious etiology.

The mother underwent induction of labor at 35 weeks 4 days, and the baby was born via uncomplicated vaginal delivery. To exclude transplacental metastases, the mother underwent dermatologic skin check, mammography, colonoscopy, and head CT, all of which were negative.

Postnatal radiographs of the baby revealed a focally dilated loop of bowel, and the patient underwent exploratory laparotomy. In the OR, nodules were noted on the small bowel serosa resulting in a bowel obstruction. Chest radiograph and CT also confirmed the presence of multiple solid lung masses. Bone scintigraphy was negative. Pathology from one of the bowel nodules revealed infantile myofibroma. Our patient has not undergone any therapeutic treatment, and follow up imaging has demonstrated continued spontaneous regression of the lung masses.

Although infantile myofibromatosis is a rare entity, it is important to include in the differential diagnosis in a fetus with multiple solid-appearing lung masses. Throughout the clinical course of these patients, imaging plays an imperative role in the assessment of these lesions.
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Authors:  Evens Ashley,  Gonzalez-gomez Ignacio,  Neville Kucera Jennifer

Keywords:  infantile myofibromatosis

Smyth Anna,  Nowik Christina,  Pugash Denise,  Rosenbaum Daniel

Final Pr. ID: Poster #: CR-003

Caudal duplication syndrome is a rare entity that involves duplication of various structures arising from the embryonic cloaca and notochord.

This presentation outlines the case of a 38-year-old G2P1 woman referred to our institution at 21 weeks gestation for anomalies detected on antenatal ultrasound. Antenatal ultrasound demonstrated sagittal duplication of the bladder, duplex phallus, a bifid scrotum and a horseshoe kidney. A subsequent fetal MRI confirmed those findings and also demonstrated apparent duplication of the colon and dysmorphic lumbosacral spine.

Following delivery, the baby passed urine via both urethras and meconium via a right-sided anus; there was a left-sided anal dimple with an imperforate anus. VCUG showed no communication between the two bladders. A colovesical fistula was demonstrated between the left bladder and colon, which is likely the redundant duplicated colon with the imperforate anus. MRI of the abdomen and pelvis showed a lipomeningocele with attempted sacral duplication, redemonstrated duplication of the pelvic organs, and confirmed absence of a left-sided rectum and sphincteric complex.

Caudal duplication syndrome is a complex malformation, the management of which often requires a multidisciplinary approach involving radiology, general surgery, urology, and neurosurgery. This case illustrates the findings of this rare entity with good correlation between fetal and postnatal imaging. It also highlights respective contributions of the various imaging modalities in guiding management, which usually entails staged surgical correction.
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Authors:  Smyth Anna,  Nowik Christina,  Pugash Denise,  Rosenbaum Daniel

Keywords:  Caudal duplication

Dawoud Malik,  Buchmann Robert

Final Pr. ID: Poster #: CR-004

We report a case of systemic juvenile xanthogranuloma affecting the liver in an 18 month old male. Read More

Authors:  Dawoud Malik,  Buchmann Robert

Keywords:  Abdomen ,  Histiocytosis

Muthiyal Sreekumar,  Kini Viswanatha,  Koshy Sheeja

Final Pr. ID: Poster #: CR-005

Inflammatory myofibroblastic tumour is a rare quasineoplastic lesion in the gatrointensitnal tract ; often present with variable and nonspecific imaging features, which may mimic other more common lesions, including malignancy. Occurrence in early infancy involving mesentery has been only sparsely reported in literature. We present such a paradigm in a 4 months old infant with clinical, radiological and histopathological features and corroborative overview of literature.
On Ultrasound abdomen, a mass lesion measuring about 6x4cm with irregular lobulated margin in the left lumbar–iliac fossa regions , involving the mesenteric planes and contigous descending colonic wall, having heterogeneous echotexture was seen . No calcification or cystic component was evident . Left kidney and spleen were seen separately . On Doppler it showed a few areas of vascularity.
On MRI ,It measured about 6.1x5.1x5.2 cm in CC, TR and AP dimensions with lobulated margins, involving the mesentery. It was heterogeously hypo intense on T1 W images and hyper intense on T2W images. On DW sequences, a few areas of restricted diffusion , predominantly along the periphery of the lesion, while the central areas showed minimal/non-restriction ; which also reflected in ADC map. On post contrast, the lesion showed moderate heterogeneous enhancement corresponding to the areas of restricted diffusion and dominant non enhancing components, suggesting areas of necrosis. Apart from contiguous colonic wall involvement , no other evidence of loco regional infiltration or metastasis was seen. Based on these, a diagnosis of Inflammatory myofibroblastic tumor was made.with differential diagnosis of non Hodgkin's lymphoma
The patient subsequently underwent laparotomy. On Histopathology, it showed myofibroblastic spindle cells and inflammatory infiltrates of lymphocytes with no evidence of nuclear pleomorphism or atypical mitosis ; suggesting the diagnosis of inflammatory myofibroblastic tumour ; which matched the MRI diagnosis. Read More

Authors:  Muthiyal Sreekumar,  Kini Viswanatha,  Koshy Sheeja

Keywords:  Inflammatory myofibroblastic tumour ,  mesentery ,  infant

Horak Richard,  Mega James,  Tanton Phillip,  Criman Erik,  Tabak Benjamin,  Rooks Veronica

Final Pr. ID: Poster #: CR-006

Fatty falciform ligament appendage torsion (FFLAT) is a rare phenomenon as there are only two reported pediatric cases of falciform ligament fatty appendage torsion in the literature. In this case, the diagnosis was established via ultrasound (US) and confirmed with computed tomography (CT). US showed an echogenic, ill-defined mass in the epigastric region that extending into the falciform ligament. CT showed the “hyperattenuating rim” sign. This report is the first reported female pediatric case of FFLAT that was diagnosed with US and CT, given a trial of analgesics, and definitively cured via minimally invasive surgical excision.

A 13-year-old female presented to the emergency department with episodic waxing and waning abdominal pain for three days. The pain had localized to the mid-epigastrium and worsened with deep inspiration. On examination, vital signs were within normal limits. Focal tenderness was elicited upon palpation of the epigastrium. Laboratory evaluation revealed a mild leukocytosis 14.2 x 109/L, normal range (3.9-10.6 x 109/L).
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Authors:  Horak Richard,  Mega James,  Tanton Phillip,  Criman Erik,  Tabak Benjamin,  Rooks Veronica

Keywords:  Torsion ,  Falciform ligament ,  Fatty appendage torsion

Tong Jane,  Luo Yu

Final Pr. ID: Poster #: CR-007

Although acute appendicitis is thought to be result from luminal obstruction of the appendix, rarely it may develop following abdominal trauma. Traumatic appendicitis is thought to occur through direct injury to the appendix, or as a response to other abdominal organ injury. On the other hand, in patients with other organ injury, some distension of the appendix can occur with surrounding free fluid secondary to trauma, mimicking appendicitis. While the clinical presentation of traumatic appendicitis is similar to that of traditional appendicitis, differentiation between reactive appendiceal changes in the setting of traumatic injury to other intra-abdominal organs is important, as the latter will not require appendectomy. We present two pediatric patients in whom following initial suspicion of acute appendicitis, ultrasonography (US) identified mildly enlarged fluid-filled and hyperemic appendix with out of proportion complex fluid, raising the suspicion of previously unsuspected abdominal trauma. Upon further examination, injury to other abdominal solid organs was discovered as the primary cause of patient's presentation and appendiceal findings were reactive to abdominal solid organ injury. In cases of suspected appendicitis, visualization of significant free fluid with dense debris on ultrasonography (US) calls for more careful examination to assess clues of other abdominal injury.
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Authors:  Tong Jane,  Luo Yu

Keywords:  reactive appendicitis ,  abdominal trauma ,  ultrasound

Noorbakhsh Abraham,  Koning Jeffrey,  Kruk Peter

Final Pr. ID: Poster #: CR-008

We report a case of a 7 year old female who presented to urology clinic due to recurrent urinary tract infections that had started 4 years ago. The patient also reported symptoms of urge incontinence and nocturnal enuresis beginning at the same time. She previously consulted an adult gynecologist, which showed no physical exam evidence of genitourinary abnormalities. An MRI of the abdomen and pelvis was also ordered at that time which reported a normal exam except for a small left renal cyst. At our institution she underwent DMSA renal scan, which was normal. She underwent a voiding cystourethrogram (VCUG), which showed no vesicoureteral reflux. However, during the VCUG, an incidental note was made of large amounts of vaginal reflux extending into the cervix, uterus, and with spillage into the peritoneal cavity presumably via the salpinges. Read More

Authors:  Noorbakhsh Abraham,  Koning Jeffrey,  Kruk Peter

Keywords:  VCUG ,  Vaginal Reflux ,  Peritoneum

Taori Abhijeet,  Shenouda Nazih

Final Pr. ID: Poster #: CR-009

Extramedullary hematopoiesis (EH) is defined as hematopoiesis occurring in organs outside of the bone marrow. It occurs in diverse conditions, including fetal development, normal immune responses, and pathological circumstances. These sites of extramedullary hematopoiesis may present as masses mimicking malignancy or produce symptoms due to pressure effects. In the setting of an existing malignancy they may appear as metastatic deposits signifying progression of disease. It is essential to confirm this due to its prognostic and treatment implications.
We report a 2-year-old little girl who presented initially with an acute history of ataxia, nystagmus, tremor, mydriasis and bruises on her left forehead. A solid left suprarenal mass was detected and a diagnosis of Stage 4 Neuroblastoma and Opsoclonus-Myoclonus syndrome was established. Subsequently she was on treatment which included chemotherapy, IVIG and stem cell transplant. On an MRI of the abdomen done a year later, a single lesion was detected in the right lobe of the liver. On subsequent short term follow up, innumerable scattered lesions were seen in the hepatic parenchyma and were thought to represent metastases. A liver biopsy showed that these hepatic lesions represented sites of extramedullary hematopoiesis.
Extramedullary hematopoiesis has been uncommonly seen in the cranium and sacrum in the setting of Neuroblastoma. We believe this is a unique presentation with extramedullary hematopoiesis presenting as solid liver masses masquerading as metastases in a known case of Neuroblastoma. Read More

Authors:  Taori Abhijeet,  Shenouda Nazih

Keywords:  Neuroblastoma ,  Extramedullary Hematopoiesis ,  Metastases

Yen Christopher,  Kukreja Kamlesh,  Masand Prakash

Final Pr. ID: Poster #: CR-010

Female conjoined throraco-omphalopagus twins were delivered via cesarean section at 35 weeks 5 days gestational age to a 38-year-old mother who received standard prenatal care. After resuscitation, the twins were transferred to the neonatal ICU, where they remained for monitoring and growth as they were assessed for potential separation.
CT angiography was performed at 3-4 months of life using a staged approach. Selective IV and oral contrast administration was used over two visits to delineate shared and non-shared structures. Most significantly, there was a single shared liver with anomalous hepatic venous drainage. Twin A had three normal caliber hepatic veins draining into a normal IVC, but a large branch of the middle hepatic vein traversed midline into Twin B and received hepatic venous drainage from Twin B via numerous anomalous vessels. Twin B had a normal IVC but three diminutive hepatic veins, thought to be due to reduced venous drainage as a result of the anomalous shared vasculature.
After multidisciplinary discussion, interventional radiology was consulted for hepatic venogram and intervention as needed. The anomalous communicating vessels were identified on hepatic venogram via Twin A femoral approach. Occlusion of the anomalous branches was achieved with serial embolization of the primary draining vessel on the Twin A side using Amplatzer vascular plugs. Successful occlusion was confirmed on venography after the final embolization procedure.
Follow-up Doppler ultrasound exams confirmed improved hepatic venous outflow in Twin B, initially with reversal of flow in the anomalous veins back toward the IVC of Twin B, followed by nonvisualization of the anomalous veins on later exams. CT angiography later showed enlarged caliber of the native Twin B hepatic veins. Following the optimization of hepatic venous outflow, the multispecialty surgical team proceeded with separation. The twins were separated at 13 months of age without complication. In addition to restoring venous outflow to allow for a successful surgical outcome, it was noted that the Amplatzer devices were used as surgical landmarks during separation for identification of shared anatomy. Through careful planning and execution, diagnostic and interventional radiology techniques played a critical role in this successful outcome. Read More

Authors:  Yen Christopher,  Kukreja Kamlesh,  Masand Prakash

Keywords:  Conjoined twins ,  Interventional Radiology ,  CT Angiography

Johnson Brittany,  Desai Sudhen,  Minifee Paul

Final Pr. ID: Poster #: CR-011

Introduction: The management of gastrobronchial fistula (GBF) is not well defined in the literature. First line management is non-operative, allowing time for the fistula to close naturally. Surgical intervention is implemented when non-operative management fails. In medically complex patients, who often fail non-operative management, surgical procedures for closure remain high risk for complications.

Case: We present a 2 year-old female with congenital esophageal atresia, duodenal atresia and annular pancreas. Beginning October 2015, she underwent multiple surgical procedures resulting in a multitude of complications. In August 2017, she transferred to our institution for management. Additional immediate operative intervention was pursued given the presence of the GBF. Despite two attempts at operative repair, the fistula recurred. Interventional radiology, consulted March 2018, developed a plan to place a percutaneous pigtail catheter in the esophagus through the dehiscent stomach wall from an external chest tube entry site, creating an iatrogenic enterocutaneous fistula (ECF). Diversion of flow from the GBF to the ECF was hypothesized to allow a conservative alternative for closure and tissue healing to occur. Initially, a 12 French (F) pigtail catheter was placed through the gastric wall into the esophageal pouch allowing the formation of the ECF tract. The patient returned for catheter downsizing to 8F three weeks later. A third intervention was performed to reposition the catheter to ensure that the tissues remained as dehydrated as possible. In May 2018, a 7 mm Cook Biodesign SIS fistula plug was placed. The 8F catheter was used to place a guide wire through the tract, then removed. The AFP bioplug, soaked in contrast to facilitate use of fluoroscopy, was placed through the mouth to seal the gastric wall. Placement was confirmed with a rigid esophagoscope and fluoroscopy. One week later, esophogram confirmed no leak. At 1 month follow-up the patient had no evidence of right pleural fluid and remains without evidence of fistula at five months. The skin site is well-healed.

Discussion: In this case, an ECF was closed with a Cook Biodesign SIS fistula plug allowing for a successful non-operative strategy after multiple failed operative revisions. The plug has FDA approval for the treatment of anal fistula, but given the positive outcome in this case, its use in esophagocutaneous fistulas may be a viable off-label option for other patients.

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Authors:  Johnson Brittany,  Desai Sudhen,  Minifee Paul

Keywords:  Gastrobronchial Fistula ,  SIS Fistula Plug

Xie Sophia,  Pfeifer Cory

Final Pr. ID: Poster #: CR-012

Leptomeningeal melanocytosis is a rare proliferation of melanocytes in the arachnoid and pia mater that presents as diffuse leptomeningeal enhancement. Findings in a rare case of this disorder are discussed along with differential considerations and diagnostic implications. Read More

Authors:  Xie Sophia,  Pfeifer Cory

Keywords:  Leptomeningeal ,  melanocytosis ,  seizure

Alazraki Adina,  Milla Sarah

Final Pr. ID: Poster #: CR-013

Ectopic thymic tissue may be found in the neck in up to 20% of the general population. Intrathyroidal thymic rest has been described as a rare entity, present in as many as 1% of children. The course of thymic migration parallels the thyroid and parathyroid glands, which explains their similar ectopic locations. While the natural history of these lesions has not been well studied, it is likely that there is involution of thymic rests with age. A few individual case reports have described the imaging features of intrathryoidal thymic rests confirmed by histopathology and flow cytometry. The aim of this case series is to raise awareness of the characteristic sonographic appearance of this entity to the radiology community. Read More

Authors:  Alazraki Adina,  Milla Sarah

Keywords:  Thymic rest ,  TiRAD ,  Thyroid

Lyon Jane,  Le Hau

Final Pr. ID: Poster #: CR-014

Inflammatory Myofibroblastic Tumor (IMT) is now considered a distinct entity and a true neoplasm within the heterogeneous group of inflammatory mass-forming tumors. It is now recognized as a fibroblastic/myofibroblastic neoplasm with intermediate biological potential. It occurs predominantly in children. Abnormalities on Chromosome 2p23 with a rearrangement of the ALK (anaplastic lymphoma kinase) locus causes abnormal tyrosine kinase receptor expression. Chromosomal abnormalities suggest a clonal origin and not just a reactive process or “pseudotumor,” as these masses have been categorized in the past. Up to sixty percent of inflammatory myofibroblastic tumors express ALK which may help establish the diagnosis of the inflammatory mass as an IMT. The masses can occur in a variety of locations and have non-specific imaging findings, which will be reviewed.

We present three cases, each in a different location:

Case 1: 7 year old female presents with chronic cough and persistent right middle lobe abnormality on chest x-ray with concern for pneumonia or inhaled foreign body. She was found to have a soft tissue mass in her right mainstem bronchus with extra-luminal extension. CT, MR and gross surgical photos of the endobronchial IMT will be presented.

Case 2: 20 month old male presents with hepatomegaly, jaundice and elevated bilirubin, alkaline phosphatase and liver function tests. Ultrasound and MR images of the pancreatic head IMT causing biliary obstruction will be presented.

Case 3: 13 year old male presents with back, leg and pelvic pain. MR, CT and gross surgical photos of the right posterior pelvic sidewall IMT, which had evidence of nerve entrapment at biopsy, will be presented.
The imaging, pathological and surgical findings from these patients, where available, will be presented and reviewed.

We suggest that the radiologist consider Inflammatory Myofibroblastic Tumor in the differential diagnosis for inflammatory and fibrous lesions in children. Read More

Authors:  Lyon Jane,  Le Hau

Keywords:  Inflammatory Myofibroblastic Tumor ,  Pediatric ,  Neoplastic