Riccardo A Superina

Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, United States

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Publications (159)620.12 Total impact

  • Henry C. Lin · Estella M. Alonso · Riccardo A. Superina · Peter F. Whitington

    No preview · Article · Dec 2015
  • Timothy B Lautz · Sonam A Shah · Riccardo A Superina
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    ABSTRACT: Two children developed hepatoblastoma concurrent with congenital portosystemic shunts (Abernethy malformations). Both underwent operative ligation of their portosystemic shunts. One received concurrent tumor resection, while the other was deemed initially unresectable and underwent biopsy followed by neoadjuvant chemotherapy. While benign hepatic masses - such as focal nodular hyperplasia and nodular regenerative hyperplasia - are common in patients with Abernethy malformations, malignant tumors have also been documented, and should always be considered in the differential diagnosis of a patient with a congenital portosystemic shunt and a hepatic mass.
    No preview · Article · Oct 2015 · Journal of pediatric gastroenterology and nutrition
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    ABSTRACT: Approaches to the management of portal hypertension (PHT) and variceal hemorrhage in pediatrics remain controversial, in large part, because they are not well informed by rigorous clinical studies. Fundamental biological and clinical differences preclude automatic application of approaches used for adults to children. On April 11-12, 2015 experts in the field convened at the first Baveno Pediatric Satellite Meeting to discuss and explore current available evidence regarding indications for MesoRex Bypass (MRB) in extrahepatic portal vein obstruction (EHPVO) and the role of primary prophylaxis of variceal hemorrhage in children. Consensus was reached regarding MRB. The vast majority of children with EHPVO will experience complications that can be prevented by successful MRB surgery. Therefore, children with EHPVO should be offered MRB for primary and secondary prophylaxis of variceal bleeding and other complications, if appropriate surgical expertise is available, if pre- and intra-operative evaluation demonstrates favorable anatomy, and if appropriate multi-disciplinary care is available for post-operative evaluation and management of shunt thrombosis or stenosis. In contrast consensus was not achieved regarding primary prophylaxis of varices. Although variceal hemorrhage is a concerning complication of PHT in children, the first bleed appears to be only rarely fatal and the associated morbidity has not been well characterized. There are few pediatric data to indicate the efficacy and safety of pharmacologic or endoscopic therapies as primary prophylaxis, or that prevention of a sentinel variceal bleed will ultimately improve survival. Therefore, no recommendation for primary prophylaxis with endoscopic variceal ligation, sclerotherapy, or nonspecific beta-blockade in children was proposed. This article is protected by copyright. All rights reserved.
    No preview · Article · Sep 2015 · Hepatology
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    ABSTRACT: Anastomotic ulcers are a known cause of anemia in children with a history of intestinal resection. Upper endoscopy and colonoscopy can be used to diagnose these ulcers; however, the area of involvement may be difficult to visualize with standard endoscopic techniques. Capsule endoscopy (CE) offers an alternative method for visualizing the small bowel in these patients. We describe a cohort of patients with short bowel syndrome (SBS) and anemia who had anastomotic ulcers detected by (CE). Retrospective chart review of patients with SBS at our institution who underwent CE for chronic gastrointestinal (GI) blood loss. Four patients who underwent a total of 6 CE procedures were identified. The underlying diagnoses included necrotizing enterocolitis (NEC) (n = 2), gastroschisis (n = 1), and jejunal atresia (n = 1). All patients had their ileocecal valves resected during previous surgeries, and had received blood transfusions within the previous several months. The median age at the time of CE was 5.5 years (range 4-14 years). Enterocolonic anastomotic ulcers were noted in the studies as a wide, flat circumferential lesion with a white base. The CE results guided a change in medical management in all 4 patients, including surgical revision of their anastomosis. CE may be a helpful adjunctive tool for detecting anastomotic ulcers in patients with SBS and chronic GI blood loss.
    No preview · Article · Mar 2015 · Journal of pediatric gastroenterology and nutrition
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    ABSTRACT: In a large cohort of children with intestinal failure (IF), we sought to determine the cumulative incidence of achieving enteral autonomy and identify patient and institutional characteristics associated with enteral autonomy. A multicenter, retrospective cohort analysis from the Pediatric Intestinal Failure Consortium was performed. IF was defined as severe congenital or acquired gastrointestinal diseases during infancy with dependence on parenteral nutrition (PN) >60 days. Enteral autonomy was defined as PN discontinuation >3 months. A total of 272 infants were followed for a median (IQR) of 33.5 (16.2-51.5) months. Enteral autonomy was achieved in 118 (43%); 36 (13%) remained PN dependent and 118 (43%) patients died or underwent transplantation. Multivariable analysis identified necrotizing enterocolitis (NEC; OR 2.42, 95% CI 1.33-4.47), care at an IF site without an associated intestinal transplantation program (OR 2.73, 95% CI 1.56-4.78), and an intact ileocecal valve (OR 2.80, 95% CI 1.63-4.83) as independent risk factors for enteral autonomy. A second model (n = 144) that included only patients with intraoperatively measured residual small bowel length found NEC (OR 3.44, 95% CI 1.36-8.71), care at a nonintestinal transplantation center (OR 6.56, 95% CI 2.53-16.98), and residual small bowel length (OR 1.04 cm, 95% CI 1.02-1.06 cm) to be independently associated with enteral autonomy. A substantial proportion of infants with IF can achieve enteral autonomy. Underlying NEC, preserved ileocecal valve, and longer bowel length are associated with achieving enteral autonomy. It is likely that variations in institutional practices and referral patterns also affect outcomes in children with IF. Copyright © 2015 Elsevier Inc. All rights reserved.
    Full-text · Conference Paper · Oct 2014

  • No preview · Article · May 2014 · Gastrointestinal Endoscopy
  • Lee M. Bass · Riccardo Superina · Stanley Kim · Saeed Mohammad

    No preview · Article · May 2014
  • Riccardo A Superina · Jean de Ville de Goyet

    No preview · Article · Dec 2013 · Journal of pediatric gastroenterology and nutrition
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    ABSTRACT: This is part two of a two-part state of the art - hepatoblastoma. International hepatoblastoma specialists were brought together to highlight advances, controversies, and future challenges in the treatment of this rare pediatric tumor. Pretreatment extent of disease (PRETEXT) is a grouping system introduced as part of the multicenter international childhood liver tumors strategy group, SIOPEL-1, study in 1990. The system has been refined over the ensuing years and has now come to be adopted for risk stratification by all of the major pediatric liver tumor multicenter trial groups. PRETEXT is being intensively studied in the current Children's Oncology Group (COG) AHEP-0731 trial in an attempt to validate interobserver reproducibility and ability to monitor response to neoadjuvant chemotherapy, and determine surgical resectability. PRETEXT is now used to identify those patients who are at risk for having an unresectable tumor and who should be referred to a liver specialty center with transplant capability early in their treatment schema. International collaborative efforts in hepatoblastoma have led to increased refinements in the use of the PRETEXT and post-treatment extent to define prognosis and surgical resectability. PRETEXT criteria which suggest a possible need for liver transplantation are discussed in detail.
    No preview · Article · Dec 2013 · Current opinion in pediatrics
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    Riccardo Superina

    Preview · Article · May 2013 · Liver Transplantation
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    ABSTRACT: Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by ~50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 ± 11.3 to 10.8 ± 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 ± 0.5 to 4.0 ± 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 ± 53.6 to 65.7 ± 9.6 μmol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.
    No preview · Article · Feb 2013 · CardioVascular and Interventional Radiology
  • Rukhmi Bhat · Timothy B Lautz · Riccardo A Superina · Robert Liem
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    ABSTRACT: Background/Purpose Extrahepatic portal vein obstruction (EHPVO) is an important cause of chronic portal hypertension in children. Although usually idiopathic in etiology, genetic and acquired thrombophilia have been implicated in EHPVO. Meso-Rex bypass is increasingly used to treat EHPVO in children. Objective The objective of this study is to assess the relationship of postoperative anticoagulation strategies and thrombophilic risk factors to the development of bypass thrombosis following the meso-Rex bypass. Methods Records of children who underwent meso-Rex bypass for EHPVO at a single institution from 1999 to 2009 were reviewed, and preoperative thrombophilia testing, perioperative anticoagulation strategies, and postoperative bypass patency based on imaging at last follow-up were examined. Results Sixty-five children with EHPVO underwent a first time meso-Rex bypass during the study period, and 9 of 65 (14 %) developed bypass thrombosis. The use of warfarin in the postoperative period was more common among children with thrombosed shunts than among those with open shunts [63 % vs. 20 %; OR, 6.5 (95 % CI, 1.3–31.5), p = 0.022]. The contribution of genetic or acquired thrombophilia to shunt thrombosis was inconclusive given variability in testing. Conclusions Choice of anticoagulation following meso-Rex bypass may affect postoperative incidence of bypass thrombosis. Role of thrombophilic risk factors in the development of shunt thrombosis remains unclear.
    No preview · Article · Feb 2013 · Journal of Gastrointestinal Surgery
  • Timothy B Lautz · Lisa A Keys · Joseph C Melvin · Joy Ito · Riccardo A Superina
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    ABSTRACT: Background: Consequences of extrahepatic portal vein obstruction (EHPVO) include variceal bleeding and hypersplenism due to portal hypertension, as well as metabolic abnormalities secondary to impaired portal venous circulation. The purpose of this study was to compare the effectiveness of meso-Rex bypass and portosystemic shunt (PSS) for reversing these symptoms in children with EHPVO. Study design: All children with idiopathic EHPVO evaluated for potential meso-Rex bypass at a single institution between 1997 and 2010 were reviewed. Portosystemic shunt was performed in patients with refractory portal hypertension when meso-Rex bypass was not technically feasible. Outcomes of meso-Rex bypass and PSS were compared, including resolution of portal hypertensive bleeding and hypersplenism, as well as changes in liver synthetic function, ammonia levels, and somatic growth. Results: Sixty-five children with EHPVO underwent successful meso-Rex bypass, while 16 required PSS. Nearly all patients experienced complete relief of variceal bleeding after meso-Rex (96%) bypass and PSS (100%). The improvements in platelet count (+82.1 ± 60.0 vs +32.4 ± 56.3 thousand/μL; p=0.004), internal normalized ratio (-0.22 ± 0.27 vs 0.01 ± 0.14; p=0.022), and serum ammonia level (-26.8 ± 36.8 vs +19.4 ± 33.1 μM/L; p=0.002) were greater after meso-Rex bypass than PSS. Among patients with below average (standard deviation z-score<0) preoperative weight for age, the improvement in weight-for-age z-score was greater after meso-Rex bypass (+0.84 ± 0.98) than PSS (+0.17 ± 0.79, p=0.044). Median duration of follow-up was 4.45 years after meso-Rex bypass and 1.8 years after PSS. Conclusions: Both meso-Rex bypass and PSS effectively relieve symptoms of portal hypertensive bleeding in children with EHPVO, although the meso-Rex better relieves hypersplenism. By restoring normal portal venous circulation, the meso-Rex bypass has additional metabolic benefits.
    No preview · Article · Nov 2012 · Journal of the American College of Surgeons
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    Timothy B Lautz · Riccardo A Superina

    Preview · Article · Aug 2012 · Cancer
  • Alexander Dzakovic · Riccardo Superina
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    ABSTRACT: Pancreatitis is becoming increasingly prevalent in children, posing new challenges to pediatric health care providers. Although some general adult treatment paradigms are applicable in the pediatric population, diagnostic workup and surgical management of acute and chronic pancreatitis have to be tailored to anatomic and pathophysiological entities peculiar to children. Nonbiliary causes of acute pancreatitis in children are generally managed nonoperatively with hydration, close biochemical and clinical observation, and early initiation of enteral feeds. Surgical intervention including cholecystectomy or endoscopic retrograde cholangiopancreatography is often required in acute biliary pancreatitis, whereas infected pancreatic necrosis remains a rare absolute indication for pancreatic debridement and drainage via open, laparoscopic, or interventional radiologic procedure. Chronic pancreatitis is characterized by painful irreversible changes of the parenchyma and ducts, which may result in or be caused by inadequate ductal drainage. A variety of surgical procedures providing drainage, denervation, resection, or a combination thereof are well established to relieve pain and preserve pancreatic function.
    No preview · Article · Aug 2012 · Seminars in Pediatric Surgery
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    ABSTRACT: To characterize the natural history of intestinal failure (IF) among 14 pediatric centers during the intestinal transplantation era. The Pediatric Intestinal Failure Consortium performed a retrospective analysis of clinical and outcome data for a multicenter cohort of infants with IF. Entry criteria included infants <12 months receiving parenteral nutrition (PN) for >60 continuous days. Enteral autonomy was defined as discontinuation of PN for >3 consecutive months. Values are presented as median (25th, 75th percentiles) or as number (%). 272 infants with a gestational age of 34 weeks (30, 36) and birth weight of 2.1 kg (1.2, 2.7) were followed for 25.7 months (11.2, 40.9). Residual small bowel length in 144 patients was 41 cm (25.0, 65.5). Diagnoses were necrotizing enterocolitis (71, 26%), gastroschisis (44, 16%), atresia (27, 10%), volvulus (24, 9%), combinations of these diagnoses (46, 17%), aganglionosis (11, 4%), and other single or multiple diagnoses (48, 18%). Prescribed medications included oral antibiotics (207, 76%), H2 blockers (187, 69%), and proton pump inhibitors (156, 57%). Enteral feeding approaches varied among centers; 19% of the cohort received human milk. The cohort experienced 8.9 new catheter-related blood stream infections per 1000 catheter days. The cumulative incidences for enteral autonomy, death, and intestinal transplantation were 47%, 27%, and 26%, respectively. Enteral autonomy continued into the fifth year after study entry. Children with IF endure significant mortality and morbidity. Enteral autonomy may require years to achieve. Improved medical, nutritional, and surgical management may reduce time on PN, mortality, and need for transplantation.
    No preview · Article · May 2012 · The Journal of pediatrics
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    Riccardo Superina
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    ABSTRACT: In distribution problems, a fleet of vehicles serves the demand of a set of customers. Each customer is typically served by a single vehicle. However, more cost effective distribution plans may exist if some customers are served by more than one vehicle, i.e., if some deliveries are split. We characterize distribution environments in which allowing split deliveries is likely to be beneficial. We show, through an empirical study, that the largest benefits are obtained when mean customer demand is a little over half the vehicle capacity and customer demand variance is relatively small.
    Preview · Article · Apr 2012 · Liver Transplantation
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    ABSTRACT: Shneider BL, Bosch J, de Franchis R, Emre SH, Groszmann RJ, Ling SC, Lorenz JM, Squires RH, Superina RA, Thompson AE, Mazariegos GV. Portal Hypertension in Children: Expert Pediatric Opinion on the Report of the Baveno V Consensus Workshop on Methodology of Diagnosis and Therapy in Portal Hypertension. Abstract: Complications of portal hypertension in children lead to significant morbidity and are a leading indication for consideration of liver transplantation. Approaches to the management of sequelae of portal hypertension are well described for adults and evidence-based approaches have been summarized in numerous meta-analyses and conferences. In contrast, there is a paucity of data to guide the management of complications of portal hypertension in children. An international panel of experts was convened on April 8, 2011 at The Children’s Hospital of Pittsburgh of UPMC to review and adapt the recent report of the Baveno V Consensus Workshop on the Methodology of Diagnosis and Therapy in Portal Hypertension to the care of children. The opinions of that expert panel are reported.
    Full-text · Article · Mar 2012 · Pediatric Transplantation
  • Timothy B Lautz · Stanley T Kim · James S Donaldson · Riccardo A Superina
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    ABSTRACT: To determine outcomes in children with extrahepatic portal vein obstruction who underwent percutaneous intervention for anastomotic stenosis after meso-Rex bypass. Eighty-six patients who underwent meso-Rex bypass between 1997 and 2009 were retrospectively reviewed, and 15 who underwent transhepatic portal venography for graft stenosis were identified. Technical success and clinical outcomes were analyzed. Nine of 15 patients (60%) with anastomotic stenosis were successfully treated by endovascular techniques and remain symptom-free with patent shunts, three (20%) underwent technically successful interventions but currently have recurrent stenosis, and three (20%) underwent failed attempts at percutaneous intervention. All three in whom percutaneous intervention failed required surgical revision, including two with near-occlusive lesions that prevented traversal of the stenosis and one who developed postprocedure shunt thrombosis. In total, patients underwent 27 endovascular procedures, including 17 balloon venoplasties, four cutting balloon venoplasties, five stent placements, and one balloon venoplasty with thrombolysis. The mean pressure gradient across the stenosis decreased from 11 mm Hg ± 3 to 5 mm Hg ± 3 (P < .001) after technically successful intervention. Platelet count improved (from 97,000/μL ± 41,000 to 165,000 ± 67,000/μL; P = .002) and ammonia levels decreased (from 40 μmol/L ± 11 to 24 μmol/L ± 13; P = .05) after intervention. Patients were followed for a median duration of 39 months after the last intervention. Percutaneous interventions allow for long-term vein graft patency and clinical resolution of symptoms in the majority of patients with anastomotic stenosis after meso-Rex bypass.
    No preview · Article · Mar 2012 · Journal of vascular and interventional radiology: JVIR
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    ABSTRACT: The meso-Rex bypass is a physiological and anatomical bypass procedure for relief of extrahepatic portal vein obstruction and restoration of mesenteric venous return to the liver. Most patients who are candidates for the bypass are children or young adults with portal hypertension and hypersplenism secondary to cavernous transformation of the portal vein. Most frequently, the bypass utilizes an autologous venous graft to connect the intrahepatic left portal vein to the infrapancreatic superior mesenteric vein (SMV) re-establishing first-pass portal perfusion. We describe the preoperative imaging of the 92 bypass candidates, the surgical anatomy as reflected in postoperative imaging, and the imaging of bypass complications at our institution. Preoperative imaging with US, CT and MR is directed to demonstrate patency and size of the left portal vein and SMV, to define the extent of cavernous transformation and splanchnic collaterals, and to assess for any associated abdominal vascular or solid organ abnormalities. Postoperative imaging is aimed at diagnosing meso-Rex bypass stenosis or occlusion and the interventional management of these complications.
    No preview · Article · Feb 2012 · Pediatric Radiology

Publication Stats

4k Citations
620.12 Total Impact Points


  • 2013-2015
    • Ann & Robert H. Lurie Children's Hospital of Chicago
      Chicago, Illinois, United States
    • Schneider Children's Medical Center of Israel
      Petah Tikva, Central District, Israel
  • 1999-2014
    • Children's Memorial Hospital
      Chicago, Illinois, United States
  • 2012
    • University of Illinois at Chicago
      Chicago, Illinois, United States
  • 2001-2012
    • Northwestern University
      • • Department of Surgery
      • • Department of Pediatrics
      Evanston, Illinois, United States
  • 2010
    • Azienda Ospedaliera San Gerardo
      Monza, Lombardy, Italy
  • 2001-2005
    • Northwestern Memorial Hospital
      • Department of Surgery
      Chicago, Illinois, United States
  • 1989-1999
    • University of Toronto
      • • Hospital for Sick Children
      • • Department of Surgery
      Toronto, Ontario, Canada
  • 1984-1999
    • SickKids
      • • Department of Surgery
      • • Division of Infectious Diseases
      Toronto, Ontario, Canada
  • 1989-1990
    • UHN: Toronto General Hospital
      Toronto, Ontario, Canada