Alkhudari Anas,  Galal Maad,  Aljabr Aljoharah

Final Pr. ID: Poster #: CR-004

In the treatment of infant hydrocephalus, ventriculoperitoneal (VP) shunts are considered the standard of care. Various complications are associated with VP shunting. Ventriculoperitoneal shunt catheter migration remains a rare but documented complication seen in one in 1000 patients who receive a VP shunt. Migration of the VP shunt into the scrotum is even more uncommon and requires surgical treatment.

We report a unique case of a 6-month-old preterm male with history of hydrocephalus treated with surgically placed VP shunt. He presented with vomiting and was found to have a right inguinoscrotal sac swelling with the tip of the catheter appreciated upon palpation of the sac and later confirmed by abdominal radiograph. After admission, the patient started to show rapid and significant improvement without significant intervention. On further imaging, the positioning of the VP shunt improved with a sufficiently reduced tip highlighting the spontaneous reduction of the distal catheter tip. Repeated imaging months later showed no recurrence.

Scrotal migrations are more common in children, and this is commonly thought to be due to an unobliterated processus vaginalis allowing the catheter tip to enter the scrotal sac. As most patients remain asymptomatic and present only after an exacerbation with co-infections, the importance of early diagnosis should not be neglected. This is crucial to avoid subsequent complications such as acute hydrocephalus, testicular torsion, and peritoneal perforation. For nearly all scrotal VP shunt migration cases, definitive treatment is surgical with manual repositioning followed by hernia repair. VP shunt migration should remain an essential differential of scrotal masses in that patient population. We also want to raise awareness to radiologists about the malposition of the VP shunt catheter and the rare ability for it to reduce and retract into appropriate positioning spontaneously. We believe that these recommendations will help improve quality of care management in pediatric hydrocephalus patients. Read More

Authors:  Alkhudari Anas , Galal Maad , Aljabr Aljoharah

Keywords:  Hydrocephalus, Ventriculoperitoneal Shunt Migration, Scrotal Sac Swelling

Collins Lee,  Pomeranz Christy,  Cohen Sara,  Baad Michael,  Kovanlikaya Arzu

Final Pr. ID: Poster #: EDU-016

Congenital portosystemic shunts represent a rare congenital malformation which diverts portal blood to the systemic circulatory system. These malformations can either be intrahepatic or extrahepatic. The intrahepatic portosystemic shunts have been classified into four types. Intrahepatic congenital portosystemic shunts demonstrates a persistent communication between the portal and hepatic venous structures, which were derived from the embryonic vitelline veins or between the vitelline and subcardinal veins. A ductus venosus, a normal structure in neonates, represents an intrahepatic congenital portosystemic shunt between the left portal vein and left hepatic vein. It typically closes between 2-17 days of postnatal life. In our institution, since April 2020, there have been five separate cases of intrahepatic congenital portosystemic shunts in newborns. We will review the imaging for each of these cases, as well as review the current literature and classification of portosystemic shunts. Read More

Authors:  Collins Lee , Pomeranz Christy , Cohen Sara , Baad Michael , Kovanlikaya Arzu

Keywords:  Portosystem Shunt, Liver, Ultrasound

Plut Domen,  Zupancic Ziva,  Mali Brajovic Senja,  Kljucevsek Tomaz,  Tomazic Mojca,  Glusic Mojca,  Homan Matjaz

Final Pr. ID: Poster #: CR-006

Congenital portosystemic venous shunts (CPVS) are rare vascular abnormalities in which the portal blood drains into a systemic vein. They are results of embryogenetic alterations during the complex development of the portal system in early gestational period. Anatomically they are classified into two groups; extrahepatic and intrahepatic. Extrahepatic shunts were first described in 1793 by Abernethy and are thus also called Abernethy malformations. Presentation of CPVS can be highly variable and consequences severe.

CASE1:
An 8 year old boy was admitted to our hospital due to severe abdominal pain. Doppler ultrasound (US) revealed an abnormal intrahepatic tubular vascular structure. The computed tomography angiography (CTA) confirmed a direct vascular connection between the left portal vein and the dilated left hepatic vein. The right portal vein was small in size, indicating main portal blood flow through the left side bypassing the liver. The treatment for this intrahepatic CPVS was embolization of the shunt. The boy has been well since the procedure.

CASE2:
A routine medical check-up in a 14 year old girl discovered abnormal liver function tests. Abdominal US showed a huge mass in the right lobe of the liver and a big mass of similar structure in the epigastrium. Portal vein was not identified. CTA showed a short main portal vein connected directly to the inferior vena cava, consistent with the Abernethy malformation. The big mass in the epigastrium was shown to be a tumor in the caudate lobe of the liver. Focal nodular hyperplasia (FNH) was considered for liver lesions due to the imaging findings and known association with the Abernethy malformation. Biopsy confirmed the diagnosis. The girl is currently waiting for liver transplantation, as it is the treatment of choice for this extrahepatic CPVS. Read More

Authors:  Plut Domen , Zupancic Ziva , Mali Brajovic Senja , Kljucevsek Tomaz , Tomazic Mojca , Glusic Mojca , Homan Matjaz

Keywords:  Congenital portosystemic venous shunt, Abernethy malformation, Intrahepatic shunt, Extrahepatic shunt

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

Oztek Murat Alp,  Parisi Marguerite,  Perez Francisco,  Phillips Grace

Final Pr. ID: Poster #: EDU-065

Ventricular shunt failures are common with as many as 40% failing by 1 year and 70% by 10 years. Approximately 15% of these shunt failures are related to shunt disconnections. The typical presenting symptoms of shunt malfunction are nonspecific and include nausea, vomiting, and altered consciousness; therefore, imaging is critical for diagnosis. Conventional radiographs can be used to identify shunt disruption although they can have limited sensitivity. Most children with suspected shunt dysfunction will also undergo CT or MR imaging to evaluate ventricular sizes. In addition, the integrity of the shunt system within the field of view on head CT can be assessed in detail; however, subtle shunt disconnections or fractures can still be difficult to detect on multiplanar CT images. Volume rendered CT images of the shunt apparatus can be used to improve the detection of subtle shunt disruptions Read More

Authors:  Oztek Murat Alp , Parisi Marguerite , Perez Francisco , Phillips Grace

Keywords:  Ventriculoperitoneal shunt, CT, Volume rendering

Dubois Josée,  Thomas-chausse Frederic,  Ghali Rafik,  Soulez Gilles

Final Pr. ID: Poster #: CR-042

Congenital portosystemic shunt is a rare vascular malformation that leads to severe complications. Two types are described: extrahepatic and intrahepatic. Extrahepatic shunt is represented by a direct communication of the portal trunk, or one of its branches of origin, to the inferior vena cava, or one of its branches.

We present two cases of extrahepatic shunt treated with stent-graft placement.

Case 1: Two y.o. male with cutaneous telangiectasias and liver calcifications on abdominal ultrasound. An extrahepatic portosystemic shunt was detected on color Doppler examination.

MR and catheter angiography revealed a large fistula between the main portal vein and the suprarenal inferior vena cava. The fistula was closed by deploying a balloon expandable stent-graft in the retrohepatic inferior vena cava. Five years later, the liver function was normalized and no complication was observed.

Case 2: Thirteen y.o. male referred for three hepatic lesions and an extrahepatic portosystemic shunt.

MR and angiography revealed a large fistula between the left portal vein and the inferior vena cava. It was closed by deploying a custom self-expandable stent-graft (Zenith, Cook Medical, Bloomington, IN) in the inferior vena cava. At five years follow-up, the patient is asymptomatic with a resolution of the liver lesions. Read More

Authors:  Dubois Josée , Thomas-chausse Frederic , Ghali Rafik , Soulez Gilles

Keywords:  Stent, Portosystemic shunt, Liver, Embolization, Children

Ramírez Tamara,  Abdeen Nishard

Final Pr. ID: Poster #: SCI-027

The optic nerve sheath diameter (ONSD) has been noted to distend in patients with increased intracranial pressure. There is relatively little data on the sensitivity of dilated optic nerve sheath on initial CT or MRI as a sign of raised Intracranial pressure (ICP) in children with ventriculoperitoneal (VP) shunt malfunction. This pilot study aims to establish if there is a significant difference in ONSD pre- and postshunt revision surgery. Read More

Authors:  Ramírez Tamara , Abdeen Nishard

Keywords:  Optic nerve sheath diameter, Intracranial pressure, Ventriculoperitoneal shunt

Hallaji Shareh,  Collins John,  Ritter Ann,  Tye Gary,  Vorona Gregory

Final Pr. ID: Poster #: EDU-080

Shunts are near-ubiquitous in the world of pediatric neuroimaging, and it is incumbent on radiologists who interpret pediatric neuroimaging to have a basic understanding of the different shunt types and components. In this educational poster we will review the appearance of a shunt with its three parts. We will correlate this to the imaging appearance of different shunt valves (programmable and non-programmable), and appropriate proximal and distal catheter positioning. We will describe the more common complications (i.e. shunt failure, subdural collections, slit ventriclular syndrome, etc.) associated with shunts in pediatric patients. The poster will also briefly highlight some of the relative advantages and disadvantages of computed tomography (CT) and magnetic resonance (MR) imaging in the assessment of hydrocephalus. Read More

Authors:  Hallaji Shareh , Collins John , Ritter Ann , Tye Gary , Vorona Gregory

Keywords:  shunts, ventriculoperitoneal

Vegunta Aishwariya

Final Pr. ID: Poster #: EDU-037

A pictorial review of imaging appearances of anatomic variations of the portal vein.
Classification and pictorial review of various congenital abnormalities of the portal vein.
Common variations and branching patterns include trifurcation of the main PV, right posterior segmental branch arising from the main PV, and right anterior segmental branch arising from the left PV. Other less common branching patterns include left PV arising from the right anterior segmental branch, duplication of PV and absent branching of PV.
Portal venous shunts may be either portosystemic shunts with abnormal communication between the portal and systemic venous systems or arterioportal shunts between portal venous system and hepatic artery.
Portosystemic shunts bypass the liver and shunt directly portal venous blood into systemic venous system.
Congenital intrahepatic portosystemic shunts are classified into four types based on Park et al. Type I, the most common type, a single vessel connects the right portal vein to the IVC. Type II in which peripheral shunt localized to one hepatic segment and single or multiple communications between branches of portal and hepatic veins. Type III with aneurysmal connection between portal and hepatic veins. Type IV with multiple communications between peripheral portal and hepatic veins in several segments.
Abernethy malformations/congenital extrahepatic portosystemic shunts can be divided into 2 major types. Type I with complete absence of intrahepatic portal venous flow and complete shunting. Type Ia with congenital absence of the portal vein and separate drainage of superior mesenteric vein and splenic vein into systemic veins. Type IIb in which superior mesenteric vein and splenic vein join to form a short extrahepatic portal vein which drains into a systemic vein. Type II with some preserved hepatic portal venous flow and partial shunting. Type IIa arising from left or right portal vein including patent ductus venosus. Type IIb arising from main portal vein. Type IIc arising from the other portal vein tributaries. Patients with both these types suffer premature mortality either due to shunting complications or other associated congenital abnormalities.
Congenital arterioportal shunts may present either as a fistula or as vascular malformations in hereditary hemorrhagic telangiectasia.
Aneurysms of the PV may represent approximately 3% of all aneurysms of the venous system. Read More

Authors:  Vegunta Aishwariya

Keywords:  Portal vein, Congenital intrahepatic portosystemic shunts, Abernethy malformations/congenital extrahepatic portosystemic shunts

Radder Shrinivas,  Razzaq Sania,  Charles Glasier,  Choudhary Arabinda,  Jayappa Sateesh,  Murphy Janice,  Ramakrishnaiah Raghu

Final Pr. ID: Poster #: EDU-080

Shunt malfunction evaluation traditionally relied on radiographs and CT head scans, but the transition to MR imaging is imperative to minimize radiation exposure. In this context, we aim to revisit the signs of variation of intracranial pressure changes on MRI in a clinical setting of suspected shunt malfunction. Read More

Authors:  Radder Shrinivas , Razzaq Sania , Charles Glasier , Choudhary Arabinda , Jayappa Sateesh , Murphy Janice , Ramakrishnaiah Raghu

Keywords:  Shunt Malfunction, Rapid Sequence Limited MRI, Revisiting MRI Signs