Imaging technologists are integral to our work.I am very pleased that, for the third year, there will be a Pediatric Imaging Technologists' Course, renamed from "Radiographers' Course to reflect its broader content.The course is again organized and directed by Laura Gruber and Christine Harris with guidance from Steve Simoneaux.The Imaging Technologists' Course is a great opportunity to learn and network.Please consider how your radiology department or group can facilitate the attendance of a deserving technologist (or more) who will surely benefit from this great program!Nashville is an extraordinary city.It is vibrant and full of life.I urge you to take time to explore what Nashville has to offer.The Omni Nashville Hotel is conveniently located close to the rich nightlife of downtown Nashville and close to many great restaurants.Be sure to try the barbecue, Prince's hot chicken and, if to your liking, local libations.Take time to listen to the music!Many choices abound and not just limited to country music.Nashville is a very progressive, diverse, inclusive, welcoming and fun city.It is a perfect environment of our meeting.We thank the people of the city of Nashville and the staff at the truly marvelous Omni Hotel Nashville for their hospitality.This meeting would not be possible if not for the extraordinary support and work of Angela Davis and Kasey O'Dea.They have been two or three steps ahead of me throughout the process.Angela and Kasey -thank you so much!!! Lastly, the SPR meeting is about people.Reacquaint yourself
Pulmonary nodules in pediatric oncology patients can present a diagnostic and treatment dilemma. Imaging findings are often nonspecific and tissue diagnosis may be required for appropriate treatment. The smaller subpleural nodules may not be visualized and cannot be palpated during video-assisted thoracoscopic surgical (VATS) resection. Preoperative localization has been beneficial in obtaining an adequate pathological specimen.
Peripherally inserted central catheters (PICCs) can lead to development of venous thrombosis and/or stenosis. The presence of venous thrombosis and/or stenosis may preclude children with chronic medical conditions from receiving lifesaving therapies, from hemodialysis in end-stage renal disease to total parenteral nutrition in short bowel syndrome. Several adult studies have found an association between PICCs and venous thrombosis and/or stenosis, but none has evaluated for this association in children.
Liver transplantation is the treatment of choice for pediatric patients suffering with complications from end-stage liver disease. Although the perioperative management of patients undergoing liver transplantation has improved significantly, post-transplantation risks of graft failure, infection, bleeding, and bowel obstruction persist.1Hackl C. Schlitt H.J. Melter M. Knoppke B. Loss M. Current developments in pediatric liver transplantation.World J Hepatol. 2015; 7: 1509-1520Crossref Scopus (43) Google Scholar Technical variant allografts from living and deceased donors account for 50% of allografts transplanted in children aged ≤5 years and are associated with an increased risk of biliary and vascular complications.2Mazariegos G.V. Garrido V. Jaskowski-Phillips S. Towbin R. Pigula F. Reyes J. Management of hepatic venous obstruction after split-liver transplantation.Pediatr Transplant. 2000; 4: 322-327Crossref PubMed Scopus (41) Google Scholar, 3Egawa H. Inomata Y. Uemoto S. Asonuma K. Kiuchi T. Okajima H. et al.Hepatic vein reconstruction in 152 living-related donor liver transplantation patients.Surgery. 1997; 121: 250-257Abstract Full Text PDF PubMed Scopus (133) Google Scholar, 4Tanaka K. Uemoto S. Tokunaga Y. Fujita S. Sano K. Nishizawa T. et al.Surgical techniques and innovations in living related liver transplantation.Ann Surg. 1993; 217: 82-91Crossref PubMed Scopus (520) Google Scholar Early recognition and rescue of allografts from perioperative complications are critical to realizing the best post-transplantation patient outcomes.5Cramm S.L. Waits S.A. Englesbe M.J. Bucuvalas J.C. Horslen S.P. Mazariegos G.V. et al.Failure to rescue as a quality improvement approach in transplantation: a first effort to evaluate this tool in pediatric liver transplantation.Transplantation. 2016; 100: 801-807Crossref PubMed Scopus (33) Google Scholar Hepatic venous outflow obstruction, although less common than portal vein or hepatic artery thrombosis, may adversely impact allograft health and function. Anatomic obstruction of the inferior vena cava (IVC) and/or hepatic vein may be caused by vascular torsion or compression (early) or by fibrosis at the surgical anastomotic site (medium to late) after liver transplantation.6Karatzas T. Lykaki-Karatzas E. Webb M. Nery J. Tsaroucha A. Demirbas A. et al.Vascular complications, treatment, and outcome following orthotopic liver transplantation.1997Google Scholar, 7Pawlak J. Grodzicki M. Leowska E. Małkowski P. Michałowicz B. Nyckowski P. et al.Vascular complications after liver transplantation.2003Google Scholar, 8Weeks S.M. Gerber D.A. Jaques P.F. Sandhu J. Johnson M.W. Fair J.H. et al.Primary Gianturco stent placement for inferior vena cava abnormalities following liver transplantation.J Vasc Interv Radiol. 2000; 11(2 Pt 1): 177-187Abstract Full Text Full Text PDF Scopus (52) Google Scholar, 9Ferro C. Andorno E. Guastavino A. Rossi U.G. Seitun S. Bovio G. et al.Endovascular treatment with primary stenting of inferior cava vein torsion following orthotopic liver transplantation with modified piggyback technique.Radiol Med. 2014; 119: 183-188Crossref PubMed Scopus (8) Google Scholar IVC obstruction leads to venous hypertension proximal to the lesion, which typically presents with a combination of signs and symptoms including ascites, lower extremity edema, hepatomegaly, pleural effusion, and graft dysfunction.9Ferro C. Andorno E. Guastavino A. Rossi U.G. Seitun S. Bovio G. et al.Endovascular treatment with primary stenting of inferior cava vein torsion following orthotopic liver transplantation with modified piggyback technique.Radiol Med. 2014; 119: 183-188Crossref PubMed Scopus (8) Google Scholar Compliant or dynamic lesions, such as vascular torsion or compression, may be associated with fluctuating symptoms owing to variability in the degree of vascular obstruction, potentially contributing to delayed or missed recognition. Treatment of post–liver transplantation IVC anastomotic complications may be pursued via surgical or transcatheter approaches. In this report, we discuss the impact of pediatric post–liver transplantation IVC stenosis, detail available treatment options, and describe a novel approach to transcatheter relief of IVC obstruction that precludes the risk of obstruction to hepatic venous egress. A 14-year-old girl with complications of portal hypertension due to cystic fibrosis–related end-stage liver disease underwent a deceased donor reduced–left lobe liver transplantation with typical bicaval and portal venous reconstruction. Following a routine early post-transplantation convalescence, she developed progressive abdominal ascites, and presented 2 months after initial hospital discharge with respiratory distress, elevated liver enzymes, and moderate ascites. She underwent paracentesis with drainage of 1200 mL of serosanguinous fluid. Analysis of liver biopsy specimens demonstrated mild acute cellular rejection. IVC venography performed in interventional radiology demonstrated a complex area of venous stenosis, felt to possibly involve the hepatic venous confluence. A pressure gradient was not assessed. Balloon venoplasty was performed, resulting in complete elimination of the lesion at low atmospheric pressure (1-2 atmospheres), suggesting a compliant nature of the stenosis (eg, vascular torsion). Over the ensuing 4 months, abdominal ascites recurred, and 2 additional paracentesis procedures were performed, with removal of 1500 mL of serous fluid at each procedure. Invasive catheter-based evaluation was reperformed at the third paracentesis procedure, which raised a concern for right atrial hypertension, prompting referral to the cardiac catheterization laboratory for complete assessment of right heart hemodynamics. On presentation for the right heart catheterization, severe ascites was noted. Invasive evaluation was notable for normal right atrial and pulmonary artery pressure but confirmed the presence of IVC stenosis, with an 8-mmHg mean pressure gradient across the IVC obstruction and nonphasic pressure tracings in the IVC and hepatic veins (Figure 1, A). Venography demonstrated a complex vascular stenosis at the level of the IVC just proximal to its entrance to the right atrium, with involvement of the hepatic venous connection to the IVC (Figure 2 and Videos 1 and 2; available at www.jpeds.com). The stenosis measured 3.5 mm at its narrowest point. There was evidence of severe contrast stasis in the distal IVC with collateralization from the infrahepatic IVC to the azygous venous system. Balloon venoplasty of the IVC lesion again supported the presence of a highly compliant vascular lesion, with elimination of the balloon waist at low pressure. The procedure was completed without further intervention.Figure 2IVC venogram in A, frontal and B, lateral projections before transcatheter intervention. There is evidence of complex and long-segment stenosis of the IVC with involvement of the hepatic venous anastomosis.View Large Image Figure ViewerDownload Hi-res image Download (PPT) A multidisciplinary discussion ensued, focusing on the potential approaches to relieving chronic IVC obstruction in the setting of a compliant vascular lesion and known caval and hepatic venous hypertension. Ultimately, the team elected to proceed with transcatheter relief of IVC stenosis in the cardiac catheterization laboratory, using a patient specific highly customized balloon-expandable vascular stent. The patient returned to the cardiac catheterization laboratory, and femoral and internal jugular venous access was obtained. Following biplane venography in multiple angles, wire position across the lesion was obtained from the femoral venous approach. A 36-mm stent (Mega LD; ev3 Endovascular, Plymouth, Minnesota) was hand-crimped onto a 10-mm × 4-cm balloon (Z-MED II; NuMED, Hopkinton, New York) and advanced to the IVC lesion via a long sheath. The stent was implanted with complete balloon/stent waist resolution at low pressure (Figure 3, A and B). The stent was deployed across the entire IVC stenosis, intentionally spanning the hepatic venous entrance into the IVC, to fully and ideally relieve the long-segment IVC stenotic lesion. We next proceeded to "customize" the stent in vivo, to facilitate unobstructed hepatic venous egress and match the stent to the patient's venous anatomy. The cranial portion of the stent extended past the stenotic area into a region of the IVC immediately proximal to the right atrium, where the cava was significantly dilated (poststenotic dilatation). In this region, the stent was flared with a 14-mm Z-MED II balloon (Figure 3, C and D). Then, to begin the process of eliminating the stent side struts that were "jailing" (or crossing) the hepatic venous egress to the IVC, we advanced a 0.014-inch coronary guide wire through a side hole in the open-cell stent and positioned in a distal hepatic vein. The stent struts (at the orifice of the hepatic vein entrance to the IVC) were then serially dilated from 2.5 mm to 14 mm in diameter, using a series of angioplasty balloons (Figure 3, E and F). In so doing, we created a customized "side hole" in the stent, thereby generating an entirely unobstructed hepatic venous egress into the stented IVC, without the presence of stent material jailing the hepatic venous confluence. After the creation of a customized side hole in the stent, the IVC portion of the stent was redilated (because the side hole creation had crushed a portion of the IVC stent posteriorly) and then further dilated to 12 mm, generating a completely unobstructed IVC (Figure 3, G and H). The final customized stent is shown in Figure 3, I and J. After all interventions, final venography demonstrated unobstructed hepatic venous egress and IVC venous flow to the right atrium, with no evidence of contrast stasis or collateral flow to the previously used azygous venous system (Figure 4 and Videos 3 and 4; available at www.jpeds.com). Hemodynamic reassessment demonstrated a trivial 2-mmHg residual mean gradient across the stented IVC with restoration of normal phasic venous flow patterns in the hepatic and infrahepatic caval veins (Figure 1, B). Ascites resolved completely, and liver enzyme levels normalized over the subsequent 2-3 weeks without the need for further paracentesis. Enoxaparin was administered for prophylactic anticoagulation, to minimize the risk of stent-related thrombus formation, until stent endothelialization was presumed to be complete at 6 months postprocedure. At the 6-month follow-up, noninvasive imaging demonstrated unobstructed IVC and hepatic veins, with no clinical recurrence of ascites or development of any stent-related complications. Enoxaparin was discontinued, and aspirin was initiated for long-term stent thromboprophylaxis. Here we describe the novel use of a highly customized balloon-expandable open-cell design endovascular stent for treating severe symptomatic post–liver transplantation IVC stenosis with infrahepatic caval and hepatic venous hypertension in a pediatric patient. Stenosis of venous anastomotic connections (hepatic veins or IVC) after liver transplantation is relatively rare, with a reported incidence of 1%-5%,8Weeks S.M. Gerber D.A. Jaques P.F. Sandhu J. Johnson M.W. Fair J.H. et al.Primary Gianturco stent placement for inferior vena cava abnormalities following liver transplantation.J Vasc Interv Radiol. 2000; 11(2 Pt 1): 177-187Abstract Full Text Full Text PDF Scopus (52) Google Scholar, 9Ferro C. Andorno E. Guastavino A. Rossi U.G. Seitun S. Bovio G. et al.Endovascular treatment with primary stenting of inferior cava vein torsion following orthotopic liver transplantation with modified piggyback technique.Radiol Med. 2014; 119: 183-188Crossref PubMed Scopus (8) Google Scholar, 10Wang S.L. Sze D.Y. Busque S. Razavi M.K. Kee S.T. Frisoli J.K. et al.Treatment of hepatic venous outflow obstruction after piggyback liver transplantation.Radiology. 2005; 236: 352-359Crossref PubMed Scopus (75) Google Scholar and vascular torsion occurs even less frequently.6Karatzas T. Lykaki-Karatzas E. Webb M. Nery J. Tsaroucha A. Demirbas A. et al.Vascular complications, treatment, and outcome following orthotopic liver transplantation.1997Google Scholar, 8Weeks S.M. Gerber D.A. Jaques P.F. Sandhu J. Johnson M.W. Fair J.H. et al.Primary Gianturco stent placement for inferior vena cava abnormalities following liver transplantation.J Vasc Interv Radiol. 2000; 11(2 Pt 1): 177-187Abstract Full Text Full Text PDF Scopus (52) Google Scholar, 9Ferro C. Andorno E. Guastavino A. Rossi U.G. Seitun S. Bovio G. et al.Endovascular treatment with primary stenting of inferior cava vein torsion following orthotopic liver transplantation with modified piggyback technique.Radiol Med. 2014; 119: 183-188Crossref PubMed Scopus (8) Google Scholar, 10Wang S.L. Sze D.Y. Busque S. Razavi M.K. Kee S.T. Frisoli J.K. et al.Treatment of hepatic venous outflow obstruction after piggyback liver transplantation.Radiology. 2005; 236: 352-359Crossref PubMed Scopus (75) Google Scholar, 11Wozney P. Zajko A.B. Bron K.M. Point S. Starzl T.E. Vascular complications after liver transplantation: a 5-year experience.AJR Am J Roentgenol. 1986; 147: 657-663Crossref PubMed Scopus (398) Google Scholar Despite the rare nature of this post-transplantation vascular complication, early recognition is important because venous obstruction is associated with significant morbidity, typically with signs of venous hypertension, including pleural effusion, ascites, graft dysfunction, peripheral edema, and abdominal pain.9Ferro C. Andorno E. Guastavino A. Rossi U.G. Seitun S. Bovio G. et al.Endovascular treatment with primary stenting of inferior cava vein torsion following orthotopic liver transplantation with modified piggyback technique.Radiol Med. 2014; 119: 183-188Crossref PubMed Scopus (8) Google Scholar Noninvasive evaluation can aid diagnosis, but IVC venography with direct measurement of a pressure gradient across the suspected lesion is the gold standard for diagnosis.8Weeks S.M. Gerber D.A. Jaques P.F. Sandhu J. Johnson M.W. Fair J.H. et al.Primary Gianturco stent placement for inferior vena cava abnormalities following liver transplantation.J Vasc Interv Radiol. 2000; 11(2 Pt 1): 177-187Abstract Full Text Full Text PDF Scopus (52) Google Scholar, 9Ferro C. Andorno E. Guastavino A. Rossi U.G. Seitun S. Bovio G. et al.Endovascular treatment with primary stenting of inferior cava vein torsion following orthotopic liver transplantation with modified piggyback technique.Radiol Med. 2014; 119: 183-188Crossref PubMed Scopus (8) Google Scholar Transcatheter therapies for anastomotic stenosis include balloon venoplasty alone or with stent implantation.2Mazariegos G.V. Garrido V. Jaskowski-Phillips S. Towbin R. Pigula F. Reyes J. Management of hepatic venous obstruction after split-liver transplantation.Pediatr Transplant. 2000; 4: 322-327Crossref PubMed Scopus (41) Google Scholar, 8Weeks S.M. Gerber D.A. Jaques P.F. Sandhu J. Johnson M.W. Fair J.H. et al.Primary Gianturco stent placement for inferior vena cava abnormalities following liver transplantation.J Vasc Interv Radiol. 2000; 11(2 Pt 1): 177-187Abstract Full Text Full Text PDF Scopus (52) Google Scholar, 9Ferro C. Andorno E. Guastavino A. Rossi U.G. Seitun S. Bovio G. et al.Endovascular treatment with primary stenting of inferior cava vein torsion following orthotopic liver transplantation with modified piggyback technique.Radiol Med. 2014; 119: 183-188Crossref PubMed Scopus (8) Google Scholar, 10Wang S.L. Sze D.Y. Busque S. Razavi M.K. Kee S.T. Frisoli J.K. et al.Treatment of hepatic venous outflow obstruction after piggyback liver transplantation.Radiology. 2005; 236: 352-359Crossref PubMed Scopus (75) Google Scholar, 12Zajko A.B. Sheng R. Bron K. Reyes J. Nour B. Tzakis A. Percutaneous transluminal angioplasty of venous anastomotic stenoses complicating liver transplantation: intermediate-term results.J Vasc Interv Radiol. 1994; 5: 121-126Abstract Full Text PDF PubMed Scopus (92) Google Scholar, 13Navarro F. Le Moine M.C. Fabre J.M. Belghiti J. Cherqui D. Adam R. et al.Specific vascular complications of orthotopic liver transplantation with preservation of the retrohepatic vena cava: review of 1361 cases.Transplantation. 1999; 68: 646-650Crossref PubMed Scopus (148) Google Scholar, 14Sze D.Y. Semba C.P. Razavi M.K. Kee S.T. Dake M.D. Endovascular treatment of hepatic venous outflow obstruction after piggyback technique liver transplantation.Transplantation. 1999; 68: 446-449Crossref PubMed Scopus (45) Google Scholar, 15Borsa J.J. Daly C.P. Fontaine A.B. Patel N.H. Althaus S.J. Hoffer E.K. et al.Treatment of inferior vena cava anastomotic stenoses with the Wallstent endoprosthesis after orthotopic liver transplantation.J Vasc Interv Radiol. 1999; 10: 17-22Abstract Full Text PDF PubMed Scopus (57) Google Scholar, 16Mathew A.T. Talbot D. Hudson M. Manas D. Rose J.D. Post-regraft supra-hepatic caval obstruction in liver transplantation: a successful outcome with expandable stents. A case report.Transpl Int. 1998; 11: 66-68Crossref PubMed Google Scholar Even though discrete fibrotic vascular lesions may respond to venoplasty alone, compliant lesions (eg, vascular torsion) necessitate the use of stent implantation to serve as scaffolding to ensure acute and sustained relief of vascular obstruction. However, doing so typically requires placement of stent material across (ie, jailing) 1 or more of the hepatic vein orifices into the IVC. This approach is suboptimal, because it provides a substrate for potential thrombus formation and/or neointimal growth in a low-flow venous system, with the potential for hepatic venous obstruction, hypertension, and devastating consequences to graft function.2Mazariegos G.V. Garrido V. Jaskowski-Phillips S. Towbin R. Pigula F. Reyes J. Management of hepatic venous obstruction after split-liver transplantation.Pediatr Transplant. 2000; 4: 322-327Crossref PubMed Scopus (41) Google Scholar Successful percutaneous stent therapy using self-expanding or premounted balloon-expandable stents has been reported in adults who have undergone liver transplantation.8Weeks S.M. Gerber D.A. Jaques P.F. Sandhu J. Johnson M.W. Fair J.H. et al.Primary Gianturco stent placement for inferior vena cava abnormalities following liver transplantation.J Vasc Interv Radiol. 2000; 11(2 Pt 1): 177-187Abstract Full Text Full Text PDF Scopus (52) Google Scholar, 10Wang S.L. Sze D.Y. Busque S. Razavi M.K. Kee S.T. Frisoli J.K. et al.Treatment of hepatic venous outflow obstruction after piggyback liver transplantation.Radiology. 2005; 236: 352-359Crossref PubMed Scopus (75) Google Scholar, 12Zajko A.B. Sheng R. Bron K. Reyes J. Nour B. Tzakis A. Percutaneous transluminal angioplasty of venous anastomotic stenoses complicating liver transplantation: intermediate-term results.J Vasc Interv Radiol. 1994; 5: 121-126Abstract Full Text PDF PubMed Scopus (92) Google Scholar, 13Navarro F. Le Moine M.C. Fabre J.M. Belghiti J. Cherqui D. Adam R. et al.Specific vascular complications of orthotopic liver transplantation with preservation of the retrohepatic vena cava: review of 1361 cases.Transplantation. 1999; 68: 646-650Crossref PubMed Scopus (148) Google Scholar, 14Sze D.Y. Semba C.P. Razavi M.K. Kee S.T. Dake M.D. Endovascular treatment of hepatic venous outflow obstruction after piggyback technique liver transplantation.Transplantation. 1999; 68: 446-449Crossref PubMed Scopus (45) Google Scholar, 15Borsa J.J. Daly C.P. Fontaine A.B. Patel N.H. Althaus S.J. Hoffer E.K. et al.Treatment of inferior vena cava anastomotic stenoses with the Wallstent endoprosthesis after orthotopic liver transplantation.J Vasc Interv Radiol. 1999; 10: 17-22Abstract Full Text PDF PubMed Scopus (57) Google Scholar, 16Mathew A.T. Talbot D. Hudson M. Manas D. Rose J.D. Post-regraft supra-hepatic caval obstruction in liver transplantation: a successful outcome with expandable stents. A case report.Transpl Int. 1998; 11: 66-68Crossref PubMed Google Scholar The use of an open-cell design stent for treating IVC stenosis, in an effort to minimize obstruction of hepatic venous egress, has been described in detail.9Ferro C. Andorno E. Guastavino A. Rossi U.G. Seitun S. Bovio G. et al.Endovascular treatment with primary stenting of inferior cava vein torsion following orthotopic liver transplantation with modified piggyback technique.Radiol Med. 2014; 119: 183-188Crossref PubMed Scopus (8) Google Scholar In that case, however, the operator did notcustomize or modify the stent, but simply relied on the open-cell design to reduce the risk of obstruction to the jailed hepatic veins. The published experience in treating IVC or hepatic venous obstruction after liver transplantation in pediatric patients is limited to a single case report. In that case, a child developed hepatic venous outflow obstruction after liver transplantation and was treated with 3 overlapping Wall stents.2Mazariegos G.V. Garrido V. Jaskowski-Phillips S. Towbin R. Pigula F. Reyes J. Management of hepatic venous obstruction after split-liver transplantation.Pediatr Transplant. 2000; 4: 322-327Crossref PubMed Scopus (41) Google Scholar Unfortunately, the patient developed an occlusive thrombus within the stent complex, which eventually necessitated open heart surgery for thrombectomy and subsequent retransplantation. This case illustrates the significant risks of thrombosis following stent placement in the pediatric population. Moreover, the presence of an isolated case report in the literature probably highlights the rarity of transcatheter treatment of pediatric post–liver transplantation IVC and hepatic venous stenosis. In the case detailed herein, we describe a novel approach to the relief of inferior caval and hepatic venous obstruction using a hand-mounted open-cell design stent with postimplantation in vivo modification to create a customized patient-specific stent. This technique was recently described in patients with congenital heart disease for treatment of complex bifurcation lesions, such as distal right ventricle–to–pulmonary artery conduit stenosis with bilateral proximal branch pulmonary artery stenosis.17Narayan H.K. Glatz A.C. Rome J.J. Bifurcating stents in the pulmonary arteries: a novel technique to relieve bilateral branch pulmonary artery obstruction.Catheter Cardiovasc Interv. 2015; 86: 714-718Crossref Scopus (7) Google Scholar The approach has been rapidly adopted by the pediatric interventional cardiology community, because it facilitates ideal treatment of a lesion that previously required open heart surgery or received inadequate relief of stenosis with standard transcatheter approaches. To date, this technique has not been widely used within the pediatric or adult interventional radiology communities and thus might not be considered when approaching the typical post–liver transplantation caval obstruction lesion. By taking a multidisciplinary collaborative approach to the discussion of the therapeutic options available in this case, we were able to translate innovative and cutting-edge techniques from the congenital heart disease population to this patient with post–liver transplantation pathology, typically under treatment of gastroenterologists, transplant surgeons, and interventional radiologists. Postimplantation stent modification by flaring (shaping) the stent and dilating the stent side holes is critical to improve vessel wall apposition and eliminate jailing of key side vessels, thereby decreasing exposure of the bare metal to the blood pool and minimizing the subsequent risk of thrombosis. This risk is not insignificant, with reports suggesting an approximate 7% incidence of thrombosis after IVC/hepatic venous stent placement in adults.18Lee J.M. Ko G.Y. Sung K.B. Gwon D.I. Yoon H.K. Lee S.G. Long-term efficacy of stent placement for treating inferior vena cava stenosis following liver transplantation.Liver Transpl. 2010; 16: 513-519Crossref PubMed Scopus (34) Google Scholar Patients with congenital heart disease (ie, Fontan physiology) who undergo bare metal stent placement in low-flow venous pathways are also considered at increased risk for thrombosis and often undergo postimplantation stent modification in an effort to minimize this risk.19Mets J.M. Bergersen L. Mayer Jr, J.E. Marshall A.C. McElhinney D.B. Outcomes of stent implantation for obstruction of intracardiac lateral tunnel Fontan pathways.Circ Cardiovasc Interv. 2013; 6: 92-100Crossref PubMed Scopus (24) Google Scholar Owing to the rarity of stent therapy for post–liver transplantation vascular complications, quantifying the exact incidence of morbidity and mortality resulting from thrombotic complications is difficult. Nonetheless, the serious morbidity associated with in-stent thrombosis in other populations20Hirono K. Ibuki K. Tomita H. Percutaneous catheter aspiration thrombectomy for the occluded stents of pulmonary artery in children with single ventricle physiology after fontan surgery.Catheter Cardiovasc Interv. 2014; 84: 1153-1156Crossref PubMed Scopus (8) Google Scholar, 21Rauch R. Sieverding L. Hofbeck M. Thrombosis of an extracardiac Fontan tunnel: combined treatment of thrombolysis and stenting.Catheter Cardiovasc Interv. 2009; 74: 917-919Crossref Scopus (6) Google Scholar should motivate providers to minimize this risk in pediatric liver transplantation recipients if possible. Finally, it is important to recognize that one of the potential limitations to the use of stent therapy to treat caval obstruction (or other vascular stenoses) in the pediatric population is the risk of a patient–stent size mismatch that may develop with ongoing somatic growth. The self-expanding stents frequently used by interventional radiologists are generally not available in smaller pediatric sizes and, when available and implanted, are incapable of later dilation to match somatic and vascular growth. In contrast, large-diameter balloon-expandable stents, such as the stent used in this case, are capable of implantation at modest diameters with subsequent balloon redilation to match somatic growth and avoid the risk of long-term patient–stent size mismatch.22Goldstein B.H. Hirsch R. Zussman M.E. Vincent J.A. Torres A.J. Coulson J. et al.Percutaneous balloon-expandable covered stent implantation for treatment of traumatic aortic injury in children and adolescents.Am J Cardiol. 2012; 110: 1541-1545Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar This approach is well established within the pediatric interventional cardiology community and could be readily translated into the pediatric post–liver transplantation population. In conclusion, IVC stenosis secondary to vascular torsion after liver transplantation is rare but causes substantial morbidity and increases the risk of graft failure. Owing to the complex hepatocaval relationship and the risk of stent-related thrombosis, the use of a patient-specific customized percutaneous stent can relieve caval and hepatic venous obstruction without putting the recipient at significant risk for subsequent thrombosis or other stent-related morbidities. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiIyY2MzMjgyYmY1MzQxNDM3NDAwOWY0MDY3ZTkzMzY4ZCIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4ODQ4NjA1fQ.N0k6LtrWWoh05fpHEbkxVKs0_lrCOliLZp-5D6PS4Z0Tpa9aDsjjjXdNokRB6J8BlXCcaFTYwvpogL7ELarw0rtiyFHzpXLdtbNjVRkNlM0EqCS8WWZV1ZNvfst8WutBRIrEi58XK78qHGrEIoQ9Is0stD6b7zW61dMwR0D9zK5i6Lej7EyB7eFptim4MgQ10779WF4ksvWnBfHJ-JeMfwDfl5QuQJY8yNCMipWcLmDvqZ6QWln_3B1vvUsKbrbcXVJGVBV_z9fcTxNdidk87-N1Hodbsfh9vOYCD-XF4Vmuyj5dHLa04scXP2EikGc7ks0y0a5-TJU-bZyD66sShA Download .mp4 (8.89 MB) Help with .mp4 files Video 1 and 2Inferior vena cava venogram in Video 1, frontal (Video 1) and Video 2, lateral (Video 2) projections, prior to transcatheter intervention. There is evidence of complex and long-segment stenosis of the IVC with involvement of the hepatic venous anastomosis. Severe contrast stasis is present in the IVC with late contrast return via the azygous system to the superior vena cava.eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiI3ZGJjYWFkYjhlZjNjN2I1NjA3ZDM2NmQyMTU0N2Y3NyIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4ODQ4NjA1fQ.hwMUPB7U93uDUnv4lVseB4wLSah3-eE19Y5P76kfSWwkK8bPJLXBvYtGdxRVjV1pgr6MkHDrYHIHLJohQeiUu6caQ2oddeu8GelPEjueEH-auhOKsydXV65WPd3dmwJqWP-R-W-KHxlfzFyQXksUwe7Zb7Xnauiq_aq7ZASPtzJZ9XhPkJ6XXy0073nMY04JbcrGaubO4VAwCL_DqSMnrTO1sM95s62Egdu2pHgraeDgNDYDuWS3mpgE_-PqherHXvUVNjkN5wjyt9r-Fbvo2ya-g2BgHd5kvHCqTEBibjsc2cqCY8f4_GZ5vz2DecTo0H7sBEOqBbhgR5POQCCZBQ Download .mp4 (9.86 MB) Help with .mp4 files Video 1 and 2Inferior vena cava venogram in Video 1, frontal (Video 1) and Video 2, lateral (Video 2) projections, prior to transcatheter intervention. There is evidence of complex and long-segment stenosis of the IVC with involvement of the hepatic venous anastomosis. Severe contrast stasis is present in the IVC with late contrast return via the azygous system to the superior vena cava. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiJlMTk2NGE4YjQzZmZlZDQ3NjA3ODk3ZTczYTAzODUwYyIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4ODQ4NjA1fQ.RWKX03vGn0gwVE5EkqlLvP_CZLJtqWCtuC-YsXAFRddUDRuiY0B-xONfIeyow05pkIxcCHNbeXwwT-Evolb2cC71Sg9pAuUSPBt7uJCOHOT3Zz4Jphm2U5KLru211GhOW6qsIrXcGK2XA6bW7NZgWug-vYFc-uwq0NniHMNpEbJ_8e2Z5igTcHVFfFI-msZsf_qhwHM4lTcs_1oYvk5i9VpXiOHlUg47d3hbfqJzhJoKljemI1kteYnuSlrMIasAo2_vTxwriPHPtHm3F86PdxneU-tceH5zm2g-wWFg4ubsAZVxXc3NdqIF2sWt5ONw9JWyH6jA8DVuHLv_3R9KnQ Download .mp4 (0.47 MB) Help with .mp4 files Video 3 and 4Inferior vena cava venogram in Video 3, frontal (Video 3) and Video 4, lateral (Video 4) projections, following transcatheter IVC stent implantation and post-implant customization. There is unobstructed and rapid IVC and hepatic venous egress to the right atrium. The customized IVC stent is apparent with openings for the proximal and distal IVC as well as the hepatic vein. Neither contrast stasis nor azygous collateralization is present.eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiJlZWMyOWIxNzYzOWQ5YzgwMjRjZTU0ZWQ5Nzk5MWY2MiIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4ODQ4NjA1fQ.Bg4M4pPMLNyj0TkqEnJOb4XjzAbUXXodmZHEy9UosOBo4hZJbufTaM4I7Rw_4JsCzsbfLfacVpDD9HeIyPQAsNTjSzys5UkrQmDfCTwvy0LU7f1eu83y9f4aCVW7Ti2nRLMVHKo-CjxMGA9mIwGfpyc8cJXMRjwyDcSE9f65QyFTmISHY3ixtS1h3kU8kADuv7DJ8IKa-Lf59tDG-LJyXAAW4sHtOcjokCtuw2G99LAYEgh91Ra658ORLZnL0G7tB0uq9N1ZkMjh3tUHIU0zlV5zjaLGUhPx0_kU60gAVgTG20aJH-69M9fIEg2NdSdmIMeZgxMJmBTDJhtCP3X9bw Download .mp4 (0.59 MB) Help with .mp4 files Video 3 and 4Inferior vena cava venogram in Video 3, frontal (Video 3) and Video 4, lateral (Video 4) projections, following transcatheter IVC stent implantation and post-implant customization. There is unobstructed and rapid IVC and hepatic venous egress to the right atrium. The customized IVC stent is apparent with openings for the proximal and distal IVC as well as the hepatic vein. Neither contrast stasis nor azygous collateralization is present.
A 17-year-old boy with heterotaxy (dextrocardia, double outlet right ventricle s/p Fontan, malrotation s/p Ladd's, dextrogastria, midline liver, and asplenia) was referred to Gastroenterology Unit for evaluation of abnormal magnetic resonance cholangiopancreatography and right upper quadrant abdominal pain. Magnetic resonance cholangiopancreatography revealed multiple hepatic nodules and dilated right-sided biliary tree without a visible connection to the normal-appearing hepatic and common bile ducts on the left. His bilirubin and transaminases were elevated. Magnetic resonance imaging with gadoxetate (Eovist) contrast was performed demonstrating hepatic nodules as shown (Fig. 1).FIGURE 1: A, Coronal magnetic resonance imaging (MRI) obtained 20 minutes after injection of gadoxetate (Eovist). Fat-suppressed 3D T1 Gradient Echo sequence (LAVA) showing a multinodular liver with nodules demonstrating both contrast uptake (yellow arrow) and lack of uptake (white arrow). The negative uptake nodule marked with an asterisk was biopsied. B, Coronal MRI (same pulse sequence but more anterior slice) showing unobstructed left liver segmental bile ducts (white arrows) forming the common bile duct (yellow arrow) which was shown to empty into a malrotated, left-sided duodenum.Insofar as endoscopic retrograde cholangiopancreatography was technically unfeasible, percutaneous cholangiography and liver biopsy were pursued, revealing 2 distinct biliary systems with the right side being dilated and draining contrast mostly to an area in the superior aspect of the liver, likely within the capsule (Fig. 2). There were at least 2 thread-like connections to the left, allowing for contrast to fill an additional biliary tree with normal-appearing ducts and gallbladder. Biopsy was consistent with well-differentiated β-catenin positive hepatic neoplasm concerning for HCC.FIGURE 2: Percutaneous transhepatic cholangiogram (PTC) showing separate biliary trees with chronic obstruction of the right bile duct system communicating with the nonobstructed left bile duct system via small caliber communicating bile ducts crossing the midline (yellow arrows). Contrast was injected through the PTC needle (labeled) into the dilated right duct system. Contrast then flowed into the left bile duct system through the small midline communicating ducts and subsequently, through a left common bile duct (CBD) and papilla, thenemptying into the malrotated, leftsided duodenum(C). A small right gall bladder and normal-sized left gall bladder were identified.Anatomic liver abnormalities are rare (1–3). As per our literature review, this case report showing 2 distinct biliary trees is novel.
Peripherally inserted central catheter (PICC) is among the most common procedures performed in children in the hospital setting. PICC insertion can be simplified with the use of a sheathed needle as an alternative to the modified Seldinger technique.
: Purpose: Platinum-based chemotherapy has been the mainstay of ovarian cancer therapy for the past three decades. More recently,poly(ADP-ribose) polymerase (PARP) inhibitors have emerged as promising agents for the treatment of ovarian cancer. However,similar to platinum, all patients eventually demonstrate tumor progression while being treated with PARP inhibitor therapy, and drug resistance is a major clinical hurdle. The BRCA1 protein is involved in homologous recombination (HR) DNA repair, and mutations in the BRCA1 gene that render the protein product dysfunctional result in cellular sensitivity to DNA damaging agents. However, therapy resistance often develops. Scope: In this proposal, we aimed to characterize N-terminal deficient BRCA1proteins contribution to DNA damage repair and their ability to confer resistance to ovarian cancer therapeutics, as well as identify novel small molecules that specifically kill N-terminal deficient BRCA1 protein expressing cells. Major findings: In the reporting period, we generated and expressed a number of BRCA1 proteins produced from downstream translation start sites that were truncated at the N-terminal region and lacked the RING domain. We show that RING deficient-BRCA1 proteins were hypomorphic, contributing to RAD51 loading, PARPi and cisplatin resistance. The mechanism we describe may not be limited to cancers with BRCA1185delAG mutations and could be relevant to multiple frameshifting 5 located BRCA1 mutations. However, mutations located after Met-297 (c.891) are unlikely to develop resistance through this mechanism, as we show that the next downstream translation start site at Met-531 (c.1593) produced a functionless protein.
BACKGROUND:Gaining access into non-dilated renal collecting systems for percutaneous nephrolithotripsy, particularly in patients with prohibitive body habitus and/or scoliosis, is often challenging using conventional techniques.OBJECTIVE:To evaluate the feasibility of cone-beam CT for percutaneous nephrostomy placement for subsequent percutaneous nephrolithotripsy in children and adolescents.MATERIALS AND METHODS:A retrospective review of percutaneous nephrostomy revealed use of cone-beam CT and 3-D guidance in 12 percutaneous nephrostomy procedures for 9 patients between 2006 and 2015. All cone-beam CT-guided percutaneous nephrostomies were for pre-lithotripsy access and all 12 were placed in non-dilated collecting systems.RESULTS:Technical success was 100%. There were no complications.CONCLUSION:Cone-beam CT with 3-D guidance is a technically feasible technique for percutaneous nephrostomy in children and adolescents, specifically for nephrolithotripsy access in non-dilated collecting systems.
The purpose of this study was to reduce pediatric doses while maintaining or improving image quality scores without removing the grid from X-ray beam. This study was approved by the Institutional Animal Care and Use Committee. Three piglets (5, 14, and 20 kg) were imaged using six different selectable detector air kerma (K-air) per frame values (100%, 70%, 50%, 35%, 25%, 17.5%) with and without the grid. Number of distal branches visualized with diagnostic confidence relative to the injected vessel defined image quality score. Five pediatric interventional radiologists evaluated all images. Image quality score and piglet K-air were statistically compared using analysis of variance and receiver operating curve analysis to define the preferred dose setting and use of grid for a visibility of 2nd and 3rd order vessel branches. Grid removal reduced both dose to subject and imaging quality by 26%. Third order branches could only be visualized with the grid present; 100% detector K-air was required for smallest pig, while 70% detector K-air was adequate for the two larger pigs. Second order branches could be visualized with grid at 17.5% detector K-air for all three pig sizes. Without the grid, 50%, 35%, and 35% detector K-air were required for smallest to largest pig, respectively. Grid removal reduces both dose and image quality score. Image quality scores can be maintained with less dose to subject with the grid in the beam as opposed to removed. Smaller anatomy requires more dose to the detector to achieve the same image quality score.
: We hypothesize that a range of common ovarian cancer predisposing germ-line BRCA1 gene mutations produce semi-functional proteins that are capable of providing PARP inhibitor resistance. Gene mutations that result in the activation of DNA end resection are likely to be required for restoration of HR DNA repair in this setting. Additionally, genetic events that stabilize mutant BRCA1 proteins may be required to avoid proteasome-mediated degradation. Our objectives are to define the BRCA1 peptide region minimally required for PARP inhibitor resistance, and discover genetic alterations that activate DNA end resection as well as mutant BRCA1 protein stabilization in ovarian carcinomas. The expression of mutant BRCA1 or novel proteins identified to be important for drug resistance will be assessed for their ability to be used as biomarkers of PARP inhibitor or platinum response. Protein expression in tumors will be assessed for their potential to serve as biomarkers that predict PARP inhibitor or platinum response.
There are a variety of available imaging modalities used for minimally invasive procedures in children, however, among the most frequently used is ultrasound (US). The advantages of US are vast and include real-time visualization, lack of ionizing radiation, and all-around versatility. US is also inexpensive, portable and widely available. In general US guided procedures in children have applications in nearly every aspect of medical therapy. Properly trained practitioners with US imaging experience and detailed knowledge of the relevant anatomy provide an invaluable service to the care of pediatric patients in many centers. This paper will discuss many of the image guided procedures that are performed in children and offer practical techniques from the collective experience of our practice at a large pediatric tertiary care center.
OBJECTIVEThe purpose of this study was to validate the hypothesis that image quality of digital subtraction angiography (DSA) in pediatrics is not impaired when using a low-dose acquisition protocol.MATERIALS AND METHODSThree piglets corresponding to common pediatric population sizes were used. DSA was performed in the aorta and renal, hepatic, and superior mesenteric arteries using both the commonly used reference standard and novel radiographic imaging noise reduction technologies to ensure pairwise radiation dose and image quality comparison. The air kerma per frame at the interventional reference point for each DSA acquisition was collected as a radiation dose measure, and image quality was evaluated by five interventional radiologists in a randomized blinded fashion using a 5-point scale.RESULTSThe mean air kerma (± SD) at the interventional reference point with the novel x-ray imaging noise reduction technology was significantly lower (1.1 ± 0.8 mGy/frame) than with the reference technology (4.2 ± 3.0 mGy/frame, p = 0.005). However, image quality was statistically similar, with average scores of 3.2 ± 0.4 and 3.1 ± 0.5 for the novel and reference technologies, respectively (p = 0.934); interrater absolute agreement was 0.77.CONCLUSIONThe DSA radiation dose for pediatrics can be reduced by a factor of four with a novel x-ray imaging noise reduction technology without deterioration of image quality.
OBJECTIVE. As patients and information flow through the imaging process, value is added step-by-step when information is acquired, interpreted, and communicated back to the referring clinician. However, radiology information systems are often plagued with communication errors and delays. This article presents theories and recommends strategies to continuously improve communication in the complex environment of modern radiology.CONCLUSION. Communication theories, methods, and systems that have proven their effectiveness in other environments can serve as models for radiology.
PURPOSE:To evaluate the technical feasibility and safety of percutaneous endovascular thrombolysis for extremity deep venous thrombosis (DVT) in children < 24 months old.MATERIALS AND METHODS:A retrospective chart review of a clinical and imaging database was performed for pediatric patients who underwent endovascular therapy for DVT between January 2010 and July 2013. Indications, techniques, technical and clinical success, and complications were reviewed. Techniques for thrombolysis included catheter-directed therapy (CDT) using alteplase infusion via a multi-side hole catheter, mechanical thrombectomy, and angioplasty. Short-term outcomes were assessed using surgical and imaging follow-up examinations for patency of the targeted vessel. Patients included 11 children (mean age, 9 mo; range, 3 wk-23 mo) who consecutively underwent endovascular thrombolysis for upper extremity (n = 6) or lower extremity (n = 5) DVT. The most common indication was preservation of venous access for future cardiac surgery or medical therapy.RESULTS:The most common risk factor was the presence of a central venous catheter (10 of 11 patients). All patients with upper extremity DVT had congenital heart disease. CDT and angioplasty were performed in all patients. Venous patency was established in all patients. A grade III (95%-100%) thrombolysis response was achieved in seven patients, and a grade II (50%-95%) thrombolysis response was achieved in four patients. A major complication of pulmonary embolism occurred in one patient with upper extremity thrombolysis and was managed by intravenous systemic alteplase and heparin. No recurrence of thrombosis was found on average follow-up of 11.8 months (range, 1-41 mo).CONCLUSIONS:Percutaneous endovascular thrombolysis for extremity DVT is safe and technically feasible in children < 24 months old.
BACKGROUND:Iodinated and gadolinium contrast agents pose some risk for certain pediatric patients, including allergic-like reactions, contrast-induced nephropathy (CIN) and nephrogenic systemic fibrosis (NSF). Digital flat-panel detectors enhance image quality during angiography and might allow use of more dilute contrast material to decrease risk of complications that might be dose-dependent, such as CIN and NSF.OBJECTIVE:To assess the maximum dilution factors for iodine- and gadolinium-based contrast agents suitable for vascular imaging with fluoroscopy and digital subtraction angiography (DSA) on digital flat-panel detectors in an animal model.MATERIALS AND METHODS:We performed selective catheterization of the abdominal aorta, renal artery and common carotid artery on a rabbit. In each vessel we performed fluoroscopy and DSA during contrast material injection using iodinated and gadolinium contrast material at 100%, 80%, 50%, 33% and 20% dilutions. An image quality score (0 to 3) was assigned by each of eight evaluators. Intracorrelation coefficient, paired t-test, one-way repeated analysis of variance, Spearman correlation and receiver operating characteristic curve analysis were applied to the data.RESULTS:Overall the image quality scores correlated linearly with dilution levels. For iodinated contrast material, the optimum cut-off level for DSA when a score of at least 2 is acceptable is above 33%; it is above 50% when a score of 3 is necessary. For gadolinium contrast material, the optimum cut-off for DSA images is above 50% when a score of at least 2 is acceptable and above 80% when a score of 3 is necessary.CONCLUSION:Knowledge of the relationship between image quality and contrast material dilution might allow a decrease in overall contrast load while maintaining appropriate image quality when using digital flat-panel detectors.
Purpose The mainstay of treatment of acute DVT is anticoagulation therapy, which only prevents further clot propagation but does not directly lead to clot lysis. Long term complications of DVT include extremity edema, hyperpigmentation, pain and ulceration collectively called post thrombotic syndrome (PTS). The most important prognostic factor in preventing development of PTS was patency and competency of extremity veins. This study examines the use of minimally invasive approach, including pharmacologic thrombolysis and mechanical thrombectomy, to treat deep venous thrombosis in the pediatric population and the subsequent clinical outcome at Cincinnati Children’s Hospital Medical Center over the last 4 years. Materials and Methods A retrospective chart review was performed in concert with clinical evaluation by hematologists during the follow up period. The Villalta score for severity of PTS was calculated on these patients. Results PTS scores were available on twenty-one patients, age ranging from 1 - 21 years (mean 14.7), over the 4 year period. A total of 47 procedures were performed. Angiojet Thrombectomy System (MedRad, Warrendale, PA) was used in 31 procedures in 19 of the patients. The Trellis System was used in 5 procedures in 4 patients. Catheter directed therapy by using infusion catheter was used in 19 patients. Stents were placed in three patients with May Thurner Syndrome. The mean follow up duration was 19.6 months (ranging 4 - 44 months). During this time, 11 patients did not develop any signs or symptoms of post thrombotic syndrome. Nine patients developed minor symptoms in the affected limb but were not classified as having post thrombotic syndrome by Villalta score ( Conclusion The limited results of this study demonstrate the ability to safely perform catheter directed thrombolysis in a pediatric population. Over the study period the rate of developing post thrombotic syndrome was very low (5%).
Brady, Rebecca C. MD; Ruth, Natasha M. MD; Minevich, Eugene A. MD; Johnson, Neil D. MBBS; Passo, Murray H. MD Author Information