Groene John,  Ong Seng,  Feinstein Kate,  Slater Bethany,  Zaritzky Mario

Final Pr. ID: Poster #: EDU-017

Purpose:
A new minimally invasive procedure, the use of magnets (Flourish™) for the treatment of esophageal atresia will be described. The invention’s background, patient selection criteria, procedure details and complications are the subject of this exhibit.

Background:
Esophageal atresia is a rare congenital defect where the proximal esophagus does not connect to the distal esophagus. Classically, this defect has been treated by surgery to reconnect the ends and reestablish esophageal continuity, however a small subset may be treated with a magnetic catheter-based system. The primary goal of this procedure is to form an anastomosis while avoiding a major thoracic surgery and its complications.
The Flourish™ device uses bullet-shaped rare earth magnets which are inserted into the upper esophagus and lower esophagus. Included in these magnets is the rare earth element neodymium, which along with iron and boron create an alloy with a strength of up to 1.2 Tesla.
When the magnets are placed in close proximity at the ends of the esophageal pouches, they will attract over several days, eventually connecting the ends of the esophagus and causing an anastomosis via pressure necrosis.

Criteria:
Selection is based on absence of tracheoesophageal fistula, esophageal gap shorter than 4 cm and a mature gastrostomy tract.

Procedure:
Under fluoroscopy, the gastric catheter is advanced superiorly through the gastrostomy to the most superior end of the distal esophageal pouch. The oral catheter is then advanced in order to bring the magnets to closest proximity. Daily radiographs are obtained to evaluate magnet positions.

Complications/outcomes:
Thirteen patients that have undergone placement of the magnetic catheter-based system at six institutions. Twelve patients had an average time to achieve anastomosis of six days and progressed to full oral feeds. The most common complication was magnetic anastomosis stenosis requiring dilatation. Currently, Flourish is approved as a Humanitarian Device Exemption (HDE).
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Authors:  Groene John , Ong Seng , Feinstein Kate , Slater Bethany , Zaritzky Mario

Keywords:  Esophageal Atresia, Atresia, Flourish

Bruno Costanza,  Minniti Salvatore

Final Pr. ID: Poster #: EDU-013

To offer a complete overview of the diagnostic hypotheses of the various fluid-containing masses which can be found in the neonatal abdomen. Read More

Authors:  Bruno Costanza , Minniti Salvatore

Keywords:  Ovarian cysts, renal dilation, duodenal atresia

Supakul Nucharin

Final Pr. ID: Poster #: EDU-017

To summarize our experience using Computed Tomography Angiography (CTA) of the liver as an imaging tool for pre-living donor liver transplantation (LDLT) evaluation in biliary atresia (BA) patients. This pictorial review will illustrate and discuss several aspects of imaging techniques and the important findings that clinician need to know before LDLT.

Key imaging findings for vascular anatomy/collateral vessels will be made, emphasizing what clinicians need to know. Correlation with intraoperative findings will also be provided.
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Authors:  Supakul Nucharin

Keywords:  Biliary atresia, LDLT, liver transplantation, CTA liver, Pediatric

Chauhan Ankita,  Maller Vijetha

Final Pr. ID: Poster #: EDU-027

Biliary atresia is a progressive, fibro-obliterative disease of the intra- and extrahepatic bile ducts in infancy. This exhibit outlines the pathophysiology, diagnostic pathways, and current and emerging management strategies for biliary atresia in the pediatric age group. Read More

Authors:  Chauhan Ankita , Maller Vijetha

Keywords:  Biliary Atresia, Kasai, HIDA

Lu Fang,  Anupindi Sudha,  Pollock Avrum,  Johnson Ann,  Adzick N,  Victoria Teresa

Final Pr. ID: Poster #: EDU-075

Duodenal atresia (DA) results from failure of recanalization of the solid core of the duodenum, usually diagnosed as the classic “double bubble”. It may come in solitary or associated with other congenital abnormalities. The aim of this educational exhibit is to describe and demonstrate common and uncommon presentations of prenatal DA and associated anomalies with emphasis on fetal MR findings. Read More

Authors:  Lu Fang , Anupindi Sudha , Pollock Avrum , Johnson Ann , Adzick N , Victoria Teresa

Keywords:  duodenal atresia, Fetal MR

Law Emily,  Luo Yu

Final Pr. ID: Poster #: CR-011

Congenital Intrathoracic Stomach (CITS) is a rare and challenging congenital anomaly, particularly when associated with a very short esophagus and gastric outlet atresia. We present a case of a neonate born at 32 weeks of gestation with complex left Congenital Diaphragmatic Hernia (CDH), where intra-operative findings revealed an intrathoracic stomach that could not be reduced to the abdomen. Post-operative fluoroscopic images demonstrated an irregular stomach in the chest, extending from the thoracic inlet to the diaphragm. The proximal esophagus was exceptionally short, measuring approximately 1 cm in length, and there was a short 0.7 cm long blind-ending tubular structure or outpouching at the inferior end of the stomach, suggestive of pyloric or duodenal atresia. This case report describes the clinical presentation, radiographic findings, and surgical management of this rare anomaly. Read More

Authors:  Law Emily , Luo Yu

Keywords:  Intrathoracic stomach, CDH, Atresia

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

Ledbetter Karyn,  Adler Elena,  Subramanyam Rajeev,  Mahmoud Mohamed,  Kline-fath Beth,  Fleck Robert

Final Pr. ID: Poster #: SCI-046

Congenital lung lesions continue to be diagnosed prenatally with increasing frequency. While the etiologies frequently encountered have been well described, there are contradictions in the literature about the prevalence of pathologies. Additionally, no gender or lobar predilection has been confidently reported. The goal of this study was to delineate the epidemiology of congenital lung lesions and their mimickers at our institution to provide comparison to published data. Read More

Authors:  Ledbetter Karyn , Adler Elena , Subramanyam Rajeev , Mahmoud Mohamed , Kline-fath Beth , Fleck Robert

Keywords:  bronchial atresia, Congenital, CPAM

Michishita Yukiko,  Miyazaki Osamu,  Imai Ayako,  Okamato Reiko,  Tsutsumi Yoshiyuki,  Miyasaka Mikiko,  Sago Haruhiko,  Kanamori Yutaka,  Nosaka Shunsuke

Final Pr. ID: Poster #: SCI-004

Esophageal atresia (EA) is a relatively rare congenital anomaly. Esophageal pouch (EP) is the only direct sign of EA on fetal MRI, but is not always seen. Distended hypopharynx (DHP) has been reported as a useful prenatal sign of EA, but as EP and DHP are both subjective assessments, the prenatal diagnosis of EA is challenging. The aim of this study was to evaluate whether quantitative measurement of distended pharynx is useful in the diagnosis of EA. Read More

Authors:  Michishita Yukiko , Miyazaki Osamu , Imai Ayako , Okamato Reiko , Tsutsumi Yoshiyuki , Miyasaka Mikiko , Sago Haruhiko , Kanamori Yutaka , Nosaka Shunsuke

Keywords:  Esophageal Atresia, fetal MRI, Quantitative

Lee Mu Sook,  Lee Mi-jung,  Kim Myung-joon,  Shin Hyun Joo

Final Pr. ID: Poster #: SCI-003

To evaluate the diagnostic value of preoperative MR cholangiography (MRCP) for the diagnosis of biliary atresia in infants with jaundice. Read More

Authors:  Lee Mu Sook , Lee Mi-jung , Kim Myung-joon , Shin Hyun Joo

Keywords:  Bilary atresia, MRCP

Ho Simon,  Sharma Priya

Final Pr. ID: Poster #: EDU-020

The aim of this exhibit is to review imaging findings of neonatal biliary disease, with emphasis on biliary atresia and conditions of surgical interest. We will present a series of cases on cholestatic diseases encountered in the neonatal period and multi-modality approach to imaging these patients.
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Authors:  Ho Simon , Sharma Priya

Keywords:  Cholestasis, Biliary Atresia

Patrick Ellen,  Dickson Paula,  Alazraki Adina,  Romero Rene

Final Pr. ID: Poster #: EDU-125

Ninety percent of biliary atresia cases occur with no associated anomalies. Ten percent of cases of biliary atresia are considered syndromic, occurring in association with a variety of congenital anomalies including heterotaxy, polysplenia, asplenia, congenital cardiac defects, intestinal malrotation, interrupted IVC, hepatic artery anomalies, and portal vein anomalies, including pre-duodenal portal vein. The incidence of hepatopulmonary syndrome is also increased in this group. Images from CT, MR, radiography, nuclear medicine, ultrasound and fluoroscopy will illustrate the variety of imaging appearances of the biliary atresia/splenic malformation syndrome. Read More

Authors:  Patrick Ellen , Dickson Paula , Alazraki Adina , Romero Rene

Keywords:  Biliary atresia, Splenic malformations

Di Puglia Elazir,  Correa Joao Antonio,  Penna Claudia

Final Pr. ID: Poster #: EDU-005 (S)

-To review the etiopathogenesis of biliary atresia;
-To review the techinique, describing and ilustratins typical and atypical sonographic findings ob biliary atresia, comparing them to the normal anatomy, to help the radiologist in the differencial diagnosisof cholestasis;
-To correlate the sonographic findings with laparoscopic cholangiography. Read More

Authors:  Di Puglia Elazir , Correa Joao Antonio , Penna Claudia

Keywords:  cholestasis, biliary atresia, ultrasound

Biscaye Stephanie,  Albertario Marco,  Guesmi Myriam,  Leloutre Beatrice,  Occelli Aurelie,  Boyer Corinne

Final Pr. ID: Poster #: CR-02 (R)

the aim of the study is to report the importance of the link between intracranial bleeding (ICB) and biliary atresia in neonates. Nowadays, the incidence of bleeding secondary to vitamin K deficiency has well-decreased, thanks to systematic vitamin K prophylaxis in neonates. However, ICB remains a severe complication of Vitamine K deficiency, with a high mortality rate and possible neurological disabilities.
Biliary atresia (BA) is a life-threatening condition in neonates, commonly diagnosed in the first 2 months of life, based on jaundice, cholestasis, pale-colored stools and dark urine. Occasionally, BA can be revealed later (25 to 65 days after birth), with severe symptoms such as intracranial, nasal or gastrointestinal bleedings. Read More

Authors:  Biscaye Stephanie , Albertario Marco , Guesmi Myriam , Leloutre Beatrice , Occelli Aurelie , Boyer Corinne

Keywords:  biliary atresia, intracranial bleeding