Schoeneberg Laura,  Bornemeier Renee,  Reemtsen Brian,  Greiten Lawrence,  Greenberg S Bruce,  Lyons Karen,  Renno Markus

Final Pr. ID: Poster #: CR-001

Limited experience with 3D print technology has been reported for surgical planning in infants with congenital heart disease. We present 5 infants with complex intracardiac anatomy for whom 3D cardiac models from gated cardiac CT angiograms facilitated surgical planning.

Case 1: Term 2-day-old infant with d-transposition of the great arteries (TGA), large anterior malalignment ventricular septal defect (VSD), and severe aortic arch hypoplasia. The 3D cardiac model confirmed feasibility of biventricular repair using a Yasui-type operation, with Norwood arch reconstruction, Rastelli VSD closure, and right ventricle to pulmonary artery (RV-PA) conduit placement.

Case 2: 2-month old, ex-34-week premature infant with double-outlet right ventricle (DORV), side-by-side great arteries, large remote predominantly-subaortic VSD, and pulmonary stenosis (PS). The 3D cardiac model highlighted the remoteness of the VSD from the outflow tracts, prompting right ventricular outflow tract patch augmentation without VSD closure when the patient developed hypercyanotic spells.

Case 3: Term 7-day-old infant with Goldenhar syndrome, D-TGA, large posterior malalignment VSD with inlet extension, and PS. The first 3D cardiac model raised concern that VSD closure would be difficult as a newborn due to VSD size, prompting placement of a BT shunt and pulmonary artery band. The second 3D model at 11 months of age demonstrated feasibility of biventricular repair with Rastelli VSD closure and RV-PA conduit placement.

Case 4: Term 2-day-old infant with 22q11.2 deletion syndrome, type B interruption of a right aortic arch, aberrant left subclavian artery, posterior malalignment VSD, small bicuspid aortic valve, and superior-inferior branch pulmonary artery relationships. The 3D cardiac model demonstrated need for VSD enlargement to enable complete repair with Yasui operation with LeCompte maneuver.

Case 5: Term 3-week-old infant with mesocardia, ventricular inversion, DORV, L-malposed great arteries, subpulmonic VSD, multilevel PS, and mitral chordal attachments to the crest of the ventricular septum. The 3D cardiac model suggested that VSD enlargement might enable a double switch operation with Rastelli VSD baffle. However, during the operation at 20 months old, mitral chordal apparatus prevented VSD enlargement, necessitating bidirectional Glenn instead. Read More

Authors:  Schoeneberg Laura,  Bornemeier Renee,  Reemtsen Brian,  Greiten Lawrence,  Greenberg S Bruce,  Lyons Karen,  Renno Markus

Keywords:  3D model ,  Congenital heart disease ,  Surgical Planning

Infante Juan

Final Pr. ID: Poster #: CR-002

INTRODUCTION
Renal artery stenosis is a common complication after transplantation. In our institution, we have unfortunately found a high rate of false-positive Doppler ultrasound and non-contrast time-of-flight MRA studies that go to invasive catheterization which turn out negative for renal artery stenosis. We present one of many of our cases which show the utility of high-quality 3D MRA using another non-contrast technique, NATIVE True-FISP, which is appears better apt at achieving adequate signal even when the acquisition plane is not perpendicular to the vessel of interest.

CASE DESCRIPTION
A 13-year-old male with a history of idiopathic dilated cardiomyopathy and end-stage renal disease from presumed ischemic injury during cardiac catheterization underwent a combined cardiac transplant and deceased donor kidney transplant. Two months after the transplant, the patient was admitted for respiratory distress, pulmonary edema, and worsening cardiac function, the latter documented by routine outpatient echocardiography.

On admission, a Doppler ultrasound showed elevated peak systolic velocity of the transplant renal artery anastomosis, up to 434 cm/sec. To confirm the sonographic suspicion of renal artery stenosis, a non-contrast 3D MRA was requested which showed tight juxta-anastomic stenosis (see Figure 1).

The interventional radiology service was consulted who brought the patient to their angiography suite on the basis of the clinical and imaging findings. A diagnostic angiogram confirmed the pre-angiographic imaging findings with nearly one-to-one correlation with the MR 3D-volume-rendered images (see Figure 2). Angioplasty of this lesion was performed, and follow-up ultrasounds up to six-months post angioplasty show interval normalization of the renal artery velocities and blood pressure. Read More

Authors:  Infante Juan

Keywords:  MRA ,  Non-contrast ,  NATIVE

Bellew Elizabeth,  Sonstegard Anna Marie,  Finelt Nika,  Goldfisher Rachelle

Final Pr. ID: Poster #: CR-004

A full-term male was born with a 7 x 7 cm subcutaneous mass with central dark blue discoloration and peripheral erythema on the right flank/buttock. Initial hip and pelvis radiograph at one day old did not show evidence of calcifications. An ultrasound demonstrated an ill-defined, heterogeneous mass with internal vascularity with invasion of underlying muscle. MRI of the abdomen and pelvis with contrast at two days of age showed a T2 hyperintense mass with mild postcontrast enhancement within the right flank with infiltration of the underlying muscles including the right psoas, iliacus, gluteal, and the left erector spinae. The differential diagnosis included: kaposiform hemangioendothelioma, rhabdomyosarcoma (RMS), tufted angioma and neuroblastoma.

Given the broad list of differential diagnoses, a core biopsy of the right buttocks was obtained at 6 days of age to further characterize the lesion. Hematoxylin and eosin stained sections demonstrated highly collagenized and sclerotic spindle cells involving the adipose tissues extending into the dermis. Immunohistochemical stained sections showed positivity to desmin, myogenin and MyoD1. Fluorescence in situ hybridization studies indicated the presence of a VGLL2 gene rearrangement. These pathologic findings were consistent with sclerosing spindle cell RMS. At 13 days old, a PET scan showed the right flank lesion with minimal to no FDG uptake, a finding which is consistent with sclerosing RMS given that this sub-type consists mainly of collagen. There were no pulmonary nodules. After multiple chemotherapy cycles, subsequent MRIs of the abdomen and pelvis showed no residual enhancing lesion. The patient will be closely followed for local recurrence.

RMS, the most common soft tissue tumor seen in children, are mesenchymal tumors of skeletal muscle and are most often seen in head and neck, but are less commonly noted on the flank. Sclerosing spindle cell RMS is a subtype often seen in childhood, but exceedingly rare in newborns. Cutaneous RMS should be considered in the differential diagnosis of a large subcutaneous lesion in a newborn. Read More

Authors:  Bellew Elizabeth,  Sonstegard Anna Marie,  Finelt Nika,  Goldfisher Rachelle

Keywords:  Rhabdomyosarcoma ,  Sclerosing Spindle Cell Rhabdomyosarcoma ,  Vascular Malformation

Stein Jill,  Browne Lorna

Final Pr. ID: Poster #: CR-005

Congenital focal lung malformations (CLM) are a heterogeneous group of lesions that have been commonly classified by their features and the presence of systemic vascular supply into bronchial atresia, congenital pulmonary airway malformation, bronchogenic cyst and bronchopulmonary sequestration, however, pathologically there is considerable overlap not recognized by this classification method. Literature has hypothesized that a common pathogenetic origin of developmental bronchial obstruction, variable in timing and extent, is responsible for the spectrum observed.

The purpose of this case report is to describe the findings of an unusual CLM with concurrent intra and extrapulmonary lesions demonstrated by pre and postnatal imaging, gross and microscopic pathology.

A 19 year-old G1P0 female presented at 34 weeks gestation for evaluation of a fetal chest mass, first identified by US at 32 weeks. Fetal US showed a large right thoracic lung lesion containing two distinct macro cysts; fetal MR demonstrated T2 hyperintense signal throughout the lesion and increased volume of the affected lung with resultant mild mediastinal shift. No systemic feeding vessel was evident.

The baby was born at 39 weeks and required intubation due to respiratory distress. Postnatal CTA showed a solid lesion right thoracic apex outlined by pleural air with systemic arterial supply from the subclavian artery, no visible airways, and a central cyst with peripheral atelectasis. There was an additional unilocular cyst with air-fluid level in the perihilar right middle lung.

Thoracotomy on day 4 described an extrapulmonary sequestration (EPS) right thoracic apex and cystic lesions in the right upper and middle lobes, all of which were resected without complication. Pathology revealed similar histology throughout all resected tissue characterized by markedly dilated proximal bronchi and parenchymal maldevelopment with immature alveolar spaces as is seen in bronchial atresia, in addition to diffuse pulmonary interstitial glycogenosis.

This case of a CLM with concurrent solid and cystic lesions supports a common pathogenetic mechanism based on developmental bronchial obstruction both with and without retention of embryonic systemic vascular communication. Prenatal classification into distinct lesions is hampered by overlapping pathological features. Characterization of vascular and airway connections and awareness of potential complications are most important to guide counseling and therapy. Read More

Authors:  Stein Jill,  Browne Lorna

Keywords:  CPAM ,  Sequestration ,  Bronchial Atresia

Mikhchi Amir,  Ryabets-lienhard Anna,  Cheung Clement,  Geffner Mitchell,  Lai Lillian

Final Pr. ID: Poster #: CR-006

Massive ovarian enlargement (without underlying mass) is a rare finding in neonates. Ovarian enlargement has been associated with insulin-resistant states such as polycystic ovarian syndrome (PCOS) and rare congenital syndromes such as infants with leprechaunism. However, the extent, severity, and radiographic findings of ovarian growth in infants with syndromes of insulin resistance have not been fully described. We report a case of severe ovarian enlargement in an infant with congenital insulin resistance.

A two-month-old born at 37 weeks via C-section with a history of intrauterine growth restriction was admitted for hyperglycemia consistent with neonatal diabetes and was subsequently diagnosed with insulin resistance syndrome (either leprechaunism or Rabson-Mendenhall syndrome) secondary to an INSR gene mutation. Insulin and proinsulin levels at 3 weeks of age were markedly elevated to 582.3 uIU/mL (2.0 – 19.6) and 674.3 pmol/L (≤ 18.8), respectively.

An abdominal ultrasound (US) performed for abdominal distension demonstrated very enlarged ovaries containing sub-centimeter follicles. Ovarian volumes were 12.8 mL and 8.7 mL on the right and left, respectively. The mean ovarian volume for this age is 1.06 mL with a standard deviation (SD) of 0.96; this would place this patient's right and left ovaries 12.2 and 8.0 SD above the mean, respectively.

CT performed at three months of age for evaluation of persistent fevers demonstrated further enlargement of the ovaries. Ovarian volumes were 106 mL and 60 mL on the right and left, which were 109 and 61 SD above the mean, respectively. A repeat US performed at four months of age showed decreasing ovarian volumes measuring 51 mL and 18 mL on the right and left, respectively (which were still 49 and 25 SD above the mean).

We postulate that this case of massive ovarian enlargement in the setting of severe insulin resistance was likely due to an insulin-mediated gonadotropin-independent mechanism, as has been previously suggested in infants with leprechaunism (perhaps mediated through an intact homologous IGF-I receptor). The ovarian enlargement seen in post-pubertal females with PCOS may have a similar pathogenesis, with resultant granulosa cell proliferation with antral follicles. Ovarian cysts have also been seen in non-insulin-resistant hyperinsulinemic infants of diabetic mothers, suggesting that high circulating serum insulin may act in the trophic manner typical of gonadotropic hormones. Read More

Authors:  Mikhchi Amir,  Ryabets-lienhard Anna,  Cheung Clement,  Geffner Mitchell,  Lai Lillian

Keywords:  Ovarian Enlargement ,  Insulin-resistance

Oliver Edward,  Gitman Olga,  Gebb Juliana,  Weiss Dana,  Canning Douglas,  Hedrick Holly,  Howell Lori,  Coleman Beverly

Final Pr. ID: Poster #: CR-007

To report the prenatal ultrasound (US) and magnetic resonance imaging (MRI) findings in a prenatally diagnosed case of closed (i.e. skin-covered) cloacal exstrophy. Read More

Authors:  Oliver Edward,  Gitman Olga,  Gebb Juliana,  Weiss Dana,  Canning Douglas,  Hedrick Holly,  Howell Lori,  Coleman Beverly

Keywords:  Abdominal Wall Defect ,  Prenatal Imaging

Richards Morgan,  Riedesel Erica,  Richer Edward

Final Pr. ID: Poster #: CR-009

5 yo male with PMHx significant only for autism presented to ED with acute abdominal pain, hematemesis, and melena. Patient was tachycardic but normotensive with Hgb 5.6 g/dL (normal 11.5 – 13.5 g/dL) requiring transfusion. Patient was also jaundiced with scleral icterus, markedly elevated liver enzymes, and direct hyperbilirubinemia. Abdominal X-ray and Abdominal US did not demonstrate significant bowel pathology. However, gallbladder was abnormal with thickened, hyperemic wall and abundant internal debris. Common bile duct was massively dilated to the level of ampulla and filled with heterogeneous, avascular debris. No cholelithiasis or mass was demonstrated. Based on US, a differential diagnosis of cholecystitis, cholangitis, or choledochal cyst complicated by hemobilia was offered. MRI demonstrated dilated central intrahepatic and extrahepatic bile ducts containing layering T1 bright, T2 dark material. On arterial post-contrast images, the right hepatic artery was immediately adjacent to dilated common hepatic duct and blush of enhancement was seen concerning for active hemorrhage into type 4b choledochal cyst. The patient proceeded to diagnostic angiography, which was unable to demonstrate a site of arterial bleeding. Biliary drain was placed across the choledochal cyst with initial drainage of bloody material followed by drainage of bile. Patient subsequently underwent open cholecystectomy. Pathology demonstrated "mucinous papillary proliferation with increased histiocytes in lamina propria, consistent with gallbladder changes in Metachromatic Leukodystrophy (MDL)." Subsequent brain MRI demonstrated classic findings of MDL. Read More

Authors:  Richards Morgan,  Riedesel Erica,  Richer Edward

Keywords:  Metachromatic Leukodystrophy ,  Gallbladder ,  Hemobilia

Lee Samantha,  Bellew Elizabeth,  Amodio John

Final Pr. ID: Poster #: CR-010

Peritoneal disease in the pediatric population is exceedingly rare. When encountered, differential diagnoses typically include infection, inflammation, and metastatic disease. However, we report a case of malignant epithelial peritoneal mesothelioma to emphasize that this primary malignancy should also be considered.

A 14 year old male with a history of growth hormone deficiency, gastroesophageal reflux disease, and asthma presented to his primary care physician with chronic abdominal pain, mild anemia, and over 20 pound weight loss in a 9 month period. Outpatient workup by gastroenterology showed elevated ESR and CRP and a single hyperplastic polyp on endoscopy. MRI enterography of the abdomen and pelvis with intravenous contrast was completed to evaluate for inflammatory bowel disease. This exam revealed a thickened omentum, hyperenhancement of the peritoneum and omentum, peritoneal nodularity, a moderate to large volume of ascites, and enhancing nodules along the superior surface of the diaphragm. The nodules were further evaluated with a dedicated CT chest, abdomen, and pelvis, which confirmed the above findings.

Differential diagnosis of an abdominal mesothelial process as above includes familial mediterreanean fever, malignancy, and infection. Laparoscopic omental and peritoneal biopsies showed mesothelial hyperplasia. Genetic testing returned positive for a CDKN2A homozygous and heterozygous deletion, confirming the diagnosis of diffuse malignant peritoneal mesothelioma.

The patient is currently under treatment with a cisplatin-based chemotherapy regimen and is being closely followed. Our case exemplifies the symptoms, imaging findings, diagnosis, and treatment associated with malignant epithelial peritoneal mesothelioma. Read More

Authors:  Lee Samantha,  Bellew Elizabeth,  Amodio John

Keywords:  Mesothelioma ,  Peritoneal Mesothelioma

Patel Parth,  Shepp Kasey,  Aribindi Haritha,  Ibrahim Muaz

Final Pr. ID: Poster #: CR-011

The ingestions of magnetic foreign objects are a common occurrence in the pediatric population. The American Association of Poison Control has documented 95,700 incidents of foreign-body ingestion in the year 2011 alone with most cases occurring in children younger than 5 years of age. In this case, we present a two-year-old male who presented to the emergency department with a four-day history of bilious vomiting. Read More

Authors:  Patel Parth,  Shepp Kasey,  Aribindi Haritha,  Ibrahim Muaz

Keywords:  jejuno-jejunal ,  fistula ,  small bowel obstruction

Zintsmaster Stephen,  Guzzardo Gianna,  O'hora Kevin

Final Pr. ID: Poster #: CR-012

Neutropenic enterocolitis (NE), commonly referred to as typhlitis (from the Greek typhlos meaning blind / closed) or ileocecal syndrome, is an increasingly common complication of neutropenic pediatric patients, with a reported incidence of 0.8% – 26%. This is especially evident as the use of aggressive chemotherapy regimens in pediatric patients increases. While typically affecting the cecum, other large and small bowel involvement has been described. Symptoms often present within 2 weeks of therapy completion, concurrently with the expected leukocytosis. We present a case of NE with typical and atypical imaging findings.

The patient was a previously healthy 7-year-old male that presented with a two-week history of daily fevers, increased fatigue, abdominal pain, and easy bruising. Flow cytometry confirmed the diagnosis of suspected B-cell Acute Lymphoblastic Leukemia/Lymphoma (ALL) and the patient was started on induction chemotherapy for High Risk ALL. On the patients last day of induction (day 7), the patient began developing worsening lower abdominal pain and bloody stool. Supine radiograph demonstrated a non-specific paucity of gas in abdomen. The patient continued to decline clinically and the physical exam also showed worsening distention with involuntary guarding. A contrast enhanced computed tomography (CT) was ordered which demonstrated NE with significant wall thickening in both the cecum and rectum. Despite ECMO and other heroic efforts the patient expired.

There is one reported case in the available English literature of neutropenic enterocolitis involving the rectum. Our case demonstrates the typical location within the cecum and ascending colon, along with the rarely identified rectal involvement. It is hypothesized that an initial intestinal injury in an already immunocompromised state, allows for an increased inflammatory response and vulnerability to opportunistic bacterial invasion. Based on reports outlining colonic wall thickening in patients with NE seen on ultrasound, there is a reported mortality rate of 60% at 10 mm or greater and only 4.2% at <10 mm. The significant wall thickening present in this case was an ominous sign. However, the lack of adequate research regarding the management NE makes it difficult to create a standardized treatment protocol. This case demonstrates a common and uncommon location of NE within the colon and further demonstrates the importance of early supportive intervention when NE is suspected. Read More

Authors:  Zintsmaster Stephen,  Guzzardo Gianna,  O'hora Kevin

Keywords:  Neutropenic Enterocolitis ,  Typhlitis ,  ALL

Portal Daniel,  Neuman Jeremy

Final Pr. ID: Poster #: CR-013

A 12 year old hispanic male with a past medical history of rectal prolapse presented to the ED from the pediatrician with a Hgb of 4.5 on outpatient lab work. The patient’s mother reports a history of constipation, staying in the bathroom for long periods of time and straining. Additionally the patient has been fatigued and pale for the last year with intermittent bloody diarrhea for the last 3 years.
Inpatient testing included defecography which showed evidence of rectal prolapse. MR enterography revealed wall thickening and hyperenhancement of the rectosigmoid colon, consistent with infectious/inflammatory etiology. Colonoscopy with biopsy was performed and histopathology results were consistent with solitary rectal ulcer syndrome.
The patient was successfully treated conservatively with dietary changes, topical medications and education about bathroom habits.

Solitary rectal ulcer syndrome (SRUS), first described in 1829, is an uncommon, benign disease characterized by clinical history, imaging (including defecography, and MR enterography), colonoscopy and diagnosed by histopathological findings. With an annual prevalence of 1 in 100,000, SRUS is most commonly found in young adults, but also seen in pediatric and geriatric populations.
Clinical presentation usually includes a history of prolonged straining, constipation, lower abdominal pain, rectal bleeding, mucous discharge and rarely rectal prolapse. Solitary rectal ulcer syndrome is actually a misnomer as only 40% of cases present with ulcers; even fewer present with a solitary ulcer. The etiology is not completely understood but is likely multifactorial.
Diagnosis of SRUS is often missed or delayed due to low clinical suspicion, inadequate rectal biopsy or failure to recognize histopathological features of the disease. It is therefore important to recognize solitary rectal ulcer syndrome and the diagnostic features and maximize the likelihood of accurately diagnosing SRUS early on. Read More

Authors:  Portal Daniel,  Neuman Jeremy

Keywords:  MR Enterography ,  Solitary Rectal Ulcer Syndrome ,  Defecography

Sams Cassandra,  Ayyala Rama

Final Pr. ID: Poster #: CR-014

Mesenteric lymphatic malformations are rare intra abdominal masses. Large mesenteric malformations can present soon after birth secondary to abdominal distention or failure to thrive. However, they can also remain clinically inapparent throughout childhood. In this series, three patients, ranging in age from 8 to 16 years, present with acute or acute on chronic abdominal pain. Subsequent imaging studies demonstrated macrocystic, mesenteric lymphatic malformations. These malformations may become painful in the setting of hemorrhage or superimposed infection. As this condition is likely not at the forefront of the clinician's mind, it is incumbent upon the radiologist to recognize the imaging findings. This case study will present a multimodality approach to the diagnosis of macrocystic, mesenteric lymphatic malformations. Read More

Authors:  Sams Cassandra,  Ayyala Rama

Keywords:  Vascular Malformation ,  Abdominal

Hyde Hannah,  Tutman Jeffrey,  Blanco Ernesto

Final Pr. ID: Poster #: CR-015

Ovarian torsion is a very rare occurrence where the ovary becomes twisted, cutting off blood supply, and placing the ovary at risk of tissue death and loss of function. Appendicitis, although more common in the pediatric population, is still a relatively uncommon condition that can have grave consequences. Appendicitis secondary to ovarian torsion, in the instance where the ovary twists on its peduncle around the body of the appendix, is not only extremely rare, but puts the patient at risks for complications and consequences for both of these rare issues. This case report will tell the story of a 15-year-old girl including her initial presentation, clinical details, imaging, post-operative notes, and prognosis. Although a very atypical finding, this case highlights the importance of time and the particular dangers when dealing with two very critical diagnoses. Read More

Authors:  Hyde Hannah,  Tutman Jeffrey,  Blanco Ernesto

Keywords:  Appendicitis ,  Torsion ,  Ovary

Dorai Raju Anand,  Chauhan Ankita,  Cohen Harris

Final Pr. ID: Poster #: CR-016

Isolated bilateral fallopian tube cyst torsion is extremely rare in the pediatric population. We have found no published reports of this entity in a child. Early diagnosis of such cases is crucial for the possibility of fallopian tube salvage surgery and the prevention of irreversible damage. Nonspecific clinical and imaging findings in a few reported unilateral cases in children often make surgical intervention mandatory for the diagnosis.

A 10-year-old girl came to the hospital for acute lower abdominal pain. Ultrasound findings demonstrated two large para-ovarian cysts, contiguous with tortuous and dilated fallopian tubes, suggesting torsion of the tubes. A clinician ordered CT did not add any new information.
The patient underwent a diagnostic laparoscopy that confirms the USG impression of the torsion of the bilateral fallopian tubes. Surgical exploration demonstrated twisting of about 1080 degrees of the hemorrhagic and necrotic right paratubal cyst and tube. The left cyst and tube were torsed at 360 degrees. Right partial salpingectomy, left paratubal cyst aspiration, and detorsion of fallopian tubes was accomplished. The uterus and ovaries were healthy.

Isolated bilateral fallopian tube torsion is extremely rare in the pediatric population with nonspecific clinical and imaging findings. Diagnosis usually requires surgery. Our study showed a case of bilateral fallopian tube torsion that occurred about bilateral paratubal cysts. Visualization of a dilated fallopian tube in the presence of normal-sized ovaries must be concerning for fallopian tube torsion, which can be secondary to a paratubal mass or cyst. Prompt early recognition and operative management of this relatively rare entity may prevent unnecessary tubal resection and improve long term fertility. Read More

Authors:  Dorai Raju Anand,  Chauhan Ankita,  Cohen Harris

Keywords:  Torsion ,  Fallopian tube ,  Paratubal cysts

Quinlan Kia,  Payne Sydney,  Primack Ilana,  Hilmes Melissa,  Singh Sudha

Final Pr. ID: Poster #: CR-017

1. Berdon syndrome, or Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS), is a rare condition with heavy morbidity and high mortality. Therefore, recognition and early diagnosis of this entity are critically important for improved patient management and family counselling. Awareness of this rare disease is a prerequisite to early recognition, whether antenatally or in the neonatal period.

2. We present two cases of Berdon syndrome at our institution. Berdon syndrome is a congenital and generally fatal disease characterized by hypoperistalsis of the gastrointestinal system, non-obstructive bladder distension, and microcolon. As of 2018, only 450 cases have been reported in literature since first described in 1976.

3. We present a logical diagnostic approach to this entity from the starting point of megacystis. Starting at megacystis helps narrow the differential diagnoses, with Eagle-Barret syndrome and posterior urethral valves being the main differential considerations. Read More

Authors:  Quinlan Kia,  Payne Sydney,  Primack Ilana,  Hilmes Melissa,  Singh Sudha

Keywords:  Megacystis ,  Berdon Syndrome ,  Prune-Belly Syndrome

Chang Santos Santiago,  Garcia Cristian,  De Barbieri Florencia

Final Pr. ID: Poster #: CR-018

Show the imaging findings in a series of 4 OHVIRA syndrome, cases reported, in our hospital in a period of 10 years (May 2009 and May2019). Read More

Authors:  Chang Santos Santiago,  Garcia Cristian,  De Barbieri Florencia

Keywords:  Ohvira ,  didelfous uterus ,  renal agenesia

Laucis Nicholas,  True-malhotra Aisha,  Leschied Jessica

Final Pr. ID: Poster #: CR-019

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm presenting as a mass in the subcutis or deep dermis in the extremities of children and young adults. Although previously regarded as ‘malignant’, due to its benign microscopic appearance and favorable prognosis, this tumor was categorized as an “intermediate tumor of uncertain differentiation” in the 2013 World Health Organization classification.

AFH is often misdiagnosed on imaging and pathology. We will present the unusual case of AFH with extensive lymphadenopathy and multiple episodes of anemia. The imaging findings will be illustrated with radiographic, ultrasound, MRI, and PET/CT exams. We will also present gross specimen and pathology images.

Our case involves a 9 year old male presenting with asymptomatic left upper arm and left chest wall masses for two years. A previous biopsy of the mass and a lymph node had been performed, showing juvenile capillary hemangioma and benign progressive germinal transformation, respectively. The patient had several prior episodes of anemia requiring multiple blood transfusions. Coagulopathy workup was negative, and it was postulated that bleeding into the mass could be the source of the anemia.

Radiographs revealed a 4.5 cm ovoid mass in the posteromedial soft tissues of the upper left arm with stable size but increasing calcific serpiginous opacifications throughout the lesion compared to two years prior. Multiple round soft tissue nodules within the left axillary region were also present.

MRI revealed a heterogeneous lesion with a calcified rim with significant susceptibility artifact likely from hemosiderin. Several enlarged adjacent lymph nodes were present with additional marked lymphadenopathy filling the left axilla abutting the brachial plexus and neurovascular bundles, extending superiorly above the acromion. The largest lymph node measured 2.8 cm within a cluster of lymphadenopathy measuring 9 x 4 x 4 cm, increased compared to MRI two years prior. PET/CT revealed increased radiotracer uptake in the mass with a max SUV of 4.5 and within the left axillary lymphadenopathy with a max SUV of 6.5.

The patient underwent surgical excision of the mass with dissection and removal of two adjacent palpable lymph nodes. The mass had a firm capsule with a red/myxoid appearance measuring 6.0 x 5.0 cm. Pathology of the mass was consistent with angiomatoid fibrous histiocytoma and pathology of the lymph nodes revealed reactive follicular hyperplasia. Read More

Authors:  Laucis Nicholas,  True-malhotra Aisha,  Leschied Jessica

Keywords:  angiomatoid fibrous histiocytoma ,  lymphadenopathy ,  mass

Poletto Erica,  Fox Evan,  Malik Archana,  Geller Evan

Final Pr. ID: Poster #: CR-020

Hypertrophic pulmonary osteoarthropathy (HPOA) is a syndrome characterized by excessive proliferation of skin and bone in the distal extremities. The classic imaging finding is symmetric, smooth periosteal reaction of the bones of the forearm or lower leg. The pathogenesis of this new bone formation is not well understood. Proposed mechanisms include growth factor release mediated by the tumor itself or the shunting of megakaryocytes through the pulmonary vasculature. These growth factors may contribute to vascular proliferation and bone formation. HPOA can be idiopathic but is more commonly secondary in patients with a variety of pulmonary disorders, congenital heart disease, and inflammatory bowel disease. HPOA is more commonly seen in adults than children. We present a case of HPOA in a patient with pleuropulmonary blastoma, a rare pediatric intrathoracic tumor.


A 3 year-old girl was diagnosed with pleuropulmonary blastoma after presenting to the emergency department with a twelve-day history of fever and leg pain. As part of a fever of unknown origin workup, chest radiographs were performed, which showed a large left lower lobe mass. Contrast-enhanced computed tomography characterized the mass as mixed cystic and solid. As part of the preoperative evaluation for osseous metastatic disease, a technetium-99m bone scan was performed, which revealed bilateral, symmetric radiotracer uptake within the ulnar and fibular shafts, and the distal humeri. Subsequent radiographs of these bones demonstrated bilateral, symmetric smooth periosteal reaction. Following surgical resection of the pleuropulmonary blastoma and completion of chemotherapy, a bone scan was repeated which showed resolution of the previously seen scintigraphic findings.

To date, HPOA has never been reported in a patient with pleuropulmonary blastoma. This case report highlights that HPOA, commonly thought of as an entity of adult lung disease, can also present in children. Read More

Authors:  Poletto Erica,  Fox Evan,  Malik Archana,  Geller Evan

Keywords:  Hypertrophic Pulmonary Osteoarthropathy ,  Pleuropulmonary Blastoma

Funes Poblete Flavia,  Mackintosh Cecilia,  Vargas Maria,  Gonzalez Veronica

Final Pr. ID: Poster #: CR-021

We report a case of 2-year-old female, twin, low birth weight (37 weeks, 1630 g - 3.59 lb), who was referred to our hospital to study an indurated nodular tumor involving her left leg, which extended from her pelvis to her left foot. Blood tests showed only an increase in alkaline phosphatase (862 mg/dl). Lower-limb X-ray was performed, which showed an extensive calcified mass with fibrillar pattern affecting dermis and deep connective tissue. MRI was also performed and showed an extensive signal alteration in muscle and dermis with hipointense signal in relation to heterotopic calcifications.
The differential diagnoses were Albright hereditary osteodystrophy (AHO), fibrodysplasia ossificans progressiva (FOP), and progressive osseous heteroplasia (POH).
AHO was ruled out because of the lack of compromise of muscle plane, and the absence of congenital malformations (such as brachydactyly, obesity, low height), hypocalcemia and hyperphosphatemia. Similarly, FOP was also ruled out since it is characterized by muscle ossification, with no dermal calcifications, and hallux malformation (also absent in this case).
The diagnosis reached was POH, which is a rare genetic (autosomal dominant inheritance) disorder characterized by progressive periarticular ossification of dermis and deep connective tissues (muscles, tendons and ligaments), with no congenital malformations. The diagnosis is built upon the clinical symptoms, imaging findings and genetic study (GNAS mutation). There is no effective treatment, with frequent progression to ankylosis. Read More

Authors:  Funes Poblete Flavia,  Mackintosh Cecilia,  Vargas Maria,  Gonzalez Veronica

Keywords:  heteroplasia ,  dermal calcification ,  calcified mass

Koroulakis Dionysios,  Reilly Brian,  Whitehead Matthew

Final Pr. ID: Poster #: CR-022

Congenital and acquired forms of temporal bone dehiscences involving the facial nerve (FN) canal, semicircular canals, and otic capsule can cause functional inner ear abnormalities and represent critical landmarks for cochlear implant surgeons. The rarest and most recently described type, cochlear-facial dehiscence (CFD), is characterized by lack of an osseous partition between the labyrinthine facial nerve canal segment and the cochlea. CFD has only been reported in adults, both by CT and histopathology. We share a case of CFD diagnosed by temporal bone CT in a pediatric patient presenting with hearing loss. This is an important condition for radiologists to be aware of when evaluating the temporal bone.

Case Report (in brief)
Our patient is a male toddler who presented at 15 months of age because he was not responding to parents’ voices. Workup revealed profound bilateral sensorineural hearing loss.
At 20 months of age, he underwent an axial high-resolution CT scan without contrast with 0.625 mm thick images of the temporal bones. Cochlear-facial dehiscence was noted bilaterally, with 0.8 mm bone defects between the labyrinthine segment of the facial nerve canal and the adjacent superolateral aspect of the cochlear mid turn (CFD). This was thought to represent a possible anatomic defect responsible for the patient’s reported sensorineural hearing loss (SNHL). The remainder of the CT examination was normal without additional areas of dehiscence.

Discussion (abbreviated):
In its normal, mature state, the facial nerve passes through a fully enveloped bony canal situated superiorly and along the lateral part of the cochlea. Inadequate facial nerve canal ossification may occur secondary to impaired epithelial-mesenchymal interactions in precursor areas at intermembranous bone ossification sites.
A presumed congenital cochlear-facial dehiscence was present in our patient bilaterally, and may have been partly or entirely responsible for his hearing loss. The defect could be from ossification failure or over-resorption of the bone. Cochlear-facial dehiscence is an important diagnosis to be primed for during the assessment of temporal bone CTs, especially in patients with findings suggesting a 3rd window phenomenon. Furthermore, it is critical to convey the presence of a CFD to the otolaryngologist prior to any consideration for cochlear implantation as it may predispose the patient to facial nerve stimulation during activation or usage of the implant. Read More

Authors:  Koroulakis Dionysios,  Reilly Brian,  Whitehead Matthew

Keywords:  dehiscence ,  cochlear

Sarma Asha,  Knake Lindsey,  Whigham Amy,  Hernanz-schulman Marta,  Anani Uchenna,  Pruthi Sumit

Final Pr. ID: Poster #: CR-023

Duplication of the pituitary gland (DPG) is an extremely rare malformation, with only 40 reported cases in the medical literature. Phenotypes range from isolated DPG with normal development to cases with additional anomalies leading to morbidity and mortality (“DPG-plus” syndrome). We describe a patient with comprehensive fetal and postnatal imaging detailing known findings of DPG-plus syndrome and previously undescribed brain and body anomalies.

An infant male was diagnosed with an obstructive oral cavity mass by fetal MRI. Postnatal neuroimaging showed a fatty oral cavity mass containing dysmorphic mandibular elements. Upon biopsy, pathological assessment showed duplication of the maxillary complex versus mesenchymal hamartoma. Other anomalies included duplicated nasal cavity, absent olfactory bulbs, duplicated sella with sellar spine, optic chiasm and tubomamillary fusion, duplicated basilar artery, persistent falcine sinus, cleft palate, and bifid tongue. In addition, there was an unusual complex brainstem and cerebellar anomaly and extensive anterior cervical vertebral clefting with anterior cervicomedullary junction myelomeningocele. Body imaging showed horseshoe pulmonary sequestration, hiatal hernia, multiple intrathoracic and intraabdominal spleens in a manner inconsistent with heterotaxy, midline liver, duplicated IVC, and vertebral anomalies. Echocardiogram showed double outlet right ventricle. Genetics evaluation revealed variants of unknown significance in CCDC39, TBX5, and ZMYND10 that were considered unlikely to be related to the observed anomalies. Due to poor neurologic prognosis, the patient was transitioned to comfort care and passed away at 4 weeks of age. Read More

Authors:  Sarma Asha,  Knake Lindsey,  Whigham Amy,  Hernanz-schulman Marta,  Anani Uchenna,  Pruthi Sumit

Keywords:  Duplicated pituitary gland plus ,  Syndrome

Reeves Benjamin,  Kadom Nadja

Final Pr. ID: Poster #: CR-024

We present a case of an 8 year old female who presented with recurrent epistaxis. An endoscopy with cauterization was performed and an adenoidal mass was found. A subsequent CT showed a well-circumscribed, hypoattenuating mass within the nasopharynx. The MRI showed a heterogeneous, T2 hyperintense lesion, with predominantly hypointense T1 signal relative to muscle, and with heterogeneous contrast enhancement. An excisional biopsy was performed, and the lesion was found to be an extraosseous chordoma. The goal of this case report is to discuss the characteristics of this relatively rare entity that is a differential consideration for nasopharyngeal mass lesions.

Chordomas are a rare type of malignant neoplasm that predominantly form in the sacrococcygeal and spheno-occipital regions. They arise from embryonic remnants of the primitive notochord, which is a primitive cell line that develops around the skull base and vertebral column. Chordomas are slow-growing, locally invasive tumors that have a low propensity for distant metastasis. They can occur in any age but are seen more commonly in adults with a peak prevalence in the 4th decade of life.

Intracranial chordomas account for 1% of all intracranial tumors. They tend to have high mortality rates due to tumor recurrence and close relation to critical structures at the skull base, such as the optic system, cavernous sinus, carotid arteries, and brainstem. Extraosseous chordomas are a rare subtype, and due to the lack of classic lytic bone changes, they can be difficult to diagnose. Extraosseous chordomas of the nasopharynx can mimic many other common tumors of the nasopharyngeal region, including non-Hodgkin lymphoma, nasopharyngeal carcinoma, and Tornwaldt cysts. When a clival sinus tract is in view, however, extraosseous chordoma should jump towards the top of the differential. Read More

Authors:  Reeves Benjamin,  Kadom Nadja

Keywords:  Chordoma ,  Nasopharynx ,  Pediatric

Xie Sophia,  Veltkamp Daniel,  Pfeifer Cory

Final Pr. ID: Poster #: CR-025

ACTA2 mutation occurs in the alpha-actin gene. Since the gene product is not expressed in brain parenchyma but is a contractile protein of smooth muscle cells that make up vascular walls, structural brain findings in this disorder are postulated to be the result of mass effect from abnormal rigid arteries during development. Presented here are typical imaging findings of ACTA2 mutation to emphasize the radiologist's role in diagnosing this disease prospectively. Read More

Authors:  Xie Sophia,  Veltkamp Daniel,  Pfeifer Cory

Keywords:  ACTA2 mutation ,  cerebrovascular disease

Riemann Monique,  Bell Denise

Final Pr. ID: Poster #: CR-026

History
15 y/o female with congenital glaucoma presents to the emergency room with right eye pain, redness and swelling. She has baseline corneal opacifications and can see light and shadows but is otherwise visually impaired. The symptoms started 2 weeks prior foreign body sensation her right eye. Antibiotic drops were prescribed but symptoms continued to worsen. A week later another topical antibiotic was added but the eye began to swell, have increased pain, and developed discharge. Her opthomalogist referred her to the ER with concern of endophthalmitis (serious intraocular infection that affects the vitreous and/or aqueous humors of the eye). Read More

Authors:  Riemann Monique,  Bell Denise

Keywords:  ultrasound ,  orbits ,  cellulitis

Walcutt Julie,  Love Terri

Final Pr. ID: Poster #: CR-027

Introduction: Acute flaccid myelitis (AFM) is a relatively new subcategory of acute flaccid paralysis affecting children, which has gained increasing attention in the medical community and media in recent years. AFM is characterized by rapid onset acute flaccid limb weakness with spinal cord lesions on magnetic resonance imaging (MRI) predominantly involving the gray matter. Cranial nerve involvement has also been described. The severity of AFM nearly always requires hospitalization for supportive care, which may include mechanical ventilation. Evidence is lacking regarding the utility of specific therapies, although patients have been treated with intravenous immune globulin, steroids, and antiviral therapy. Unfortunately, though neurological improvement may occur, the majority of patients are left with persistent deficits.

We present the imaging findings of three children with varying presentations and outcomes of acute flaccid myelitis treated at a tertiary care children’s hospital.

Case 1: An 11-year-old girl developed severe upper extremity weakness requiring weeks of inpatient rehabilitation with plan for evaluation for nerve transfer. MRI showed abnormal increased T2 signal of nearly the entire spinal cord, most notable in the cervical spinal cord.

Case 2: A 9-year-old boy positive for Enterovirus presented with bilateral upper and lower extremity weakness, whose symptoms ultimately resolved. MRI of the brain and full spine were normal except for a thoracic spinal cord syrinx, which was felt to be incidental.

Case 3: A 2-year-old girl with a history of acute lymphoblastic leukemia developed bilateral upper extremity weakness and lower extremity areflexia during a hospitalization for an acute viral illness, complicated by chronic ventilator-dependent respiratory failure. MRI demonstrated enhancing T2 signal in the brainstem and cervical and thoracic spinal cord.

Discussion: Acute flaccid myelitis is an important pediatric neurological disorder of rapid clinical onset frequently resulting in persistent neurological deficits. Radiologists serve an important role in establishing the diagnosis by identifying this disease’s characteristic MRI findings. Read More

Authors:  Walcutt Julie,  Love Terri

Keywords:  weakness ,  imaging

Oztek Murat Alp,  Wright Jason,  Perez Francisco,  Khalatbari Hedieh,  Ishak Gisele

Final Pr. ID: Poster #: CR-028

Aseptic or chemical meningitis is an inflammation of the meninges without an infectious cause. It is a diagnosis of exclusion which is reached only in the appropriate clinical context after a careful search for bacterial or viral pathogens is negative. Aseptic meningitis following transsphenoidal resection of craniopharyngioma has been reported in the neurosurgical literature with an incidence of up to 3%; however, it remains unfamiliar in the radiology literature and there have been only a few case reports describing associated imaging findings. The chemical meningitis is thought to be caused by the release of craniopharyngioma fluid which contains inflammatory cholesterol crystals. Clinical and CSF findings can be identical to bacterial meningitis except for the absence of identifiable bacterial or viral pathogens. Complications can include vasospasm, stroke, and death. The purpose of this report is to describe two cases of aseptic meningitis following craniopharyngioma resection with the hope of improving awareness among pediatric radiologists. Read More

Authors:  Oztek Murat Alp,  Wright Jason,  Perez Francisco,  Khalatbari Hedieh,  Ishak Gisele

Keywords:  Craniopharyngioma ,  Meningitis ,  MRI

Ben-david Eliel,  Zharkov Elena,  Pais Adi,  Kasirer Yair,  Bin-nun Alona

Final Pr. ID: Poster #: CR-029

A novel 1T MRI scanner, previously compared to a 1.5T Siemens scanner, was placed in our NICU. The 5-gauss safety line is inside the magnet, rendering standard magnetic safety precautions unnecessary and allowing maintaining ongoing critical care. Scans included near-term equivalent stable neonates and neonates in an acute / sub-acute setting of disease. Through the following cases we present the capabilities of the 1T in-NICU scanner to evaluate the neonatal brain throughout different phases of injury.

Case 1–Chronic. A 28 5/7 week infant born to mother with chorioamnionitis, developed sepsis. Cranial US showed bilateral IVH Grade III (L) / IV (R) on DOL 2. At DOL 49, ventriculomegaly and porencephalic cysts were detected on US. MRI at term equivalent age demonstrated the post hemorrhagic cystic degeneration and ventriculomegaly. Follow up MRI was performed at 4 months of age due to increasing head circumference. Presence of prior MRI images increased diagnostic confidence.

Case 2–Sub-acute. A term infant with moderate encephalopathy treated with therapeutic hypothermia, after absent fetal movements for two days. Complicated NICU course, with multi-system organ failure, seizures and severe hypoglycemia. MRI scan on DOL 14 showed punctate rim-T1 hyperintense foci with a hypointense center, opposite T2 signal and restricted diffusion, in the centrum semi-ovale and periventricular white matter. Sub-acute hemorrhage with suspected ischemic etiology was the working diagnosis.

Case 3–Acute. A term infant delivered by stat C-section for non-reassuring fetal heart rate and low Apgar scores. Complicated NICU course, including multisystem organ failure, seizures, and burst suppression on cerebral monitoring. MRI scan on DOL 6 showed diffuse abnormal cortical T1 and white-matter T2 signal. Restricted diffusion was seen diffusely in a posterior distribution. Diffuse hypoxic ischemic injury was diagnosed.

Case 4–Hyper-Acute. A 31 week infant was delivered by stat C-section after major maternal trauma, with extremely severe hypovolemic shock. An MRI scan was performed at 8 hours of life, to determine direction of care while intubated and on vasopressor support. Extra-axial and intraventricular hemorrhage were seen, as well as diffuse restricted diffusion. The scan assisted family and physicians to reach a decision regarding discontinuing of care. Read More

Authors:  Ben-david Eliel,  Zharkov Elena,  Pais Adi,  Kasirer Yair,  Bin-nun Alona

Keywords:  Neonate ,  MRI ,  Brain

Foust Alexandra,  Voss Stephan

Final Pr. ID: Poster #: CR-030

PET/CT plays an important role in assessing response to therapy in patients undergoing treatment for Hodgkin’s lymphoma (HL). A negative PET/CT following completion of chemotherapy has a high negative predictive value (>95%) for disease progression, relapse, or recurrence. Thus treatment decisions, including the decision to pursue radiation therapy or to alter chemotherapy regimens, are often made based on PET/CT results. Given the treatment implications, obtaining accurate PET/CT results is of the utmost importance. Read More

Authors:  Foust Alexandra,  Voss Stephan

Keywords:  Abnormal FDG uptake in white fat ,  gluconeogenesis ,  Hodgkin lymphoma therapy implications

Chen Jennifer,  Milla Sarah,  Alazraki Adina

Final Pr. ID: Poster #: CR-031

Patients with Von Hippel Lindau are predisposed to develop neuroendocrine tumors (NET) such as pheochromocytoma. These tumors are known to be somatostatin receptor positive and therefore can be imaged with specific radiotracers that target these receptors like ¹¹¹In-octreotide and ⁶⁸Ga-Dotatate. In addition to NET’s, 80% of these patients will also develop hemangioblastomas in the brain and spinal cord which have been shown to harbor somatostatin receptor subtypes including SSTR-2a, one of the target receptors for DOTATATE. We present a case of a teenager with suspected NET in the adrenal gland who underwent ⁶⁸Ga-DOTATATE PET/CT. The PET confirmed uptake in the adrenal lesion, but also highlighted a focus of uptake in the spine. Correlative MRI of the spine demonstrates classic imaging features of hemangioblastoma. This finding has not previously been described in the published literature in children.
This case report will discuss the mechanism and utility of ⁶⁸Ga-Dotatate PET/CT and emphasize the manifestations of Von Hippel Lindau disease. We will review the normal pattern of Dotatate uptake and the significance of somatostatin receptors in neoplasms in the VHL patient population. Read More

Authors:  Chen Jennifer,  Milla Sarah,  Alazraki Adina

Keywords:  hemangioblastoma ,  dotatate ,  von hippel lindau

Binkovitz Larry,  Sutton Samuel

Final Pr. ID: Poster #: CR-032

To present the first PET/CT and PET/MRI imaging findings of acase of multiple myeloma, MM, in a pediatric patient. Read More

Authors:  Binkovitz Larry,  Sutton Samuel

Keywords:  Plasmacytoma ,  PET/CT ,  PET/MR