R A Superina

Northwestern University, Evanston, IL, United States

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Publications (126)477.32 Total impact

  • 2014 American Academy of Pediatrics National Conference and Exhibition; 10/2014
  • Riccardo A Superina, Jean de Ville de Goyet
    Journal of pediatric gastroenterology and nutrition 12/2013; · 2.18 Impact Factor
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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.
    Current opinion in pediatrics 12/2013; · 2.01 Impact Factor
  • Riccardo Superina
    Liver Transplantation 05/2013; 19(5). · 3.94 Impact Factor
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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.
    CardioVascular and Interventional Radiology 02/2013; · 2.09 Impact Factor
  • 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.
    Journal of Gastrointestinal Surgery 02/2013; · 2.36 Impact Factor
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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±33.1μM/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.
    Journal of the American College of Surgeons 11/2012; · 4.50 Impact Factor
  • 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.
    Seminars in Pediatric Surgery 08/2012; 21(3):266-71. · 2.40 Impact Factor
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    Timothy B Lautz, Riccardo A Superina
    Cancer 07/2012; 118(16):4094-5. · 5.20 Impact Factor
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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.
    The Journal of pediatrics 05/2012; 161(4):723-728.e2. · 4.02 Impact Factor
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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.
    Liver Transplantation 04/2012; 18(4):389-90. · 3.94 Impact Factor
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    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.
    Pediatric Transplantation 03/2012; 16(5):426-37. · 1.50 Impact Factor
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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.
    Journal of vascular and interventional radiology: JVIR 03/2012; 23(3):377-83. · 1.81 Impact Factor
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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.
    Pediatric Radiology 02/2012; 42(2):220-32; quiz 271-2. · 1.57 Impact Factor
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    ABSTRACT: An international symposium convened September 9-11, 2010, in Chicago to present the state of the art and science of the multidisciplinary care of intestinal failure in children. Medical and surgical management of the child with intestinal failure was presented with a focus on the importance of multidisciplinary intestinal failure management. Issues of timing of referral and benefit risk analysis for intestine "rehabilitation" and transplant were presented. Areas of opportunity such as increased donor recovery, improvement of long-term transplant outcomes, optimization of immune monitoring, and quality-of-life outcomes were reviewed.
    Transplantation 11/2011; 92(11):1173-80. · 3.78 Impact Factor
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    ABSTRACT: The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes. Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression). KPE was performed in 244 infants (54% female; mean age: 65 ± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection. Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.
    Annals of surgery 08/2011; 254(4):577-85. · 7.90 Impact Factor
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    ABSTRACT: Liver transplantation is increasingly advocated as primary surgical therapy for children with hepatoblastoma involving 3 or 4 sectors of the liver after neoadjuvant chemotherapy. This study evaluated the results of nontransplant hepatectomy in children who might otherwise have been considered for liver transplantation. All children who underwent resection at a single institution from 1998 to 2009 for POST-TEXT IV or centrally located POST-TEXT III hepatoblastoma after neoadjuvant chemotherapy were reviewed. Fourteen children (7 boys) with a median age of 8 months at diagnosis met study criteria. Pulmonary metastases in 3 patients were resected in 2 and resolved with chemotherapy in 1 patient. Preoperative grouping after neoadjuvant chemotherapy was POST-TEXT IV in 3 patients and POST-TEXT III in 11 patients. Thirteen of 14 (93%) children who underwent aggressive resection despite being potential candidates for primary transplantation were alive and tumor-free with a median follow-up of 57 months. Observed survival rates at 1, 2, and 5 years were 93%, 91%, and 88% respectively. Event-free survival rates at 1, 2, and 5 years were 93%, 91%, and 75%, respectively. Excellent survival (93%) was obtained with aggressive resection in children with POST-TEXT III and IV hepatoblastoma meeting criteria for transplant referral. The 1 death occurred in a patient with unfavorable small cell histology. These children should be managed at institutions experienced in both advanced pediatric hepatobiliary surgery and transplantation. Operative exploration was frequently required to ultimately determine which tumors can be resected and which require transplantation.
    Cancer 05/2011; 117(9):1976-83. · 5.20 Impact Factor
  • Timothy B Lautz, Alex Dzakovic, Riccardo A Superina
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    ABSTRACT: A 3-year-old girl with idiopathic extrahepatic portal vein obstruction undergoing mesenterico-left portal vein bypass (MLPVB), or Rex shunt, for recurrent variceal bleeding was intraoperatively discovered to have duplicated round ligaments and Rex recessi. The left and right portal veins connected to their respective round ligaments but had minimal communication within the liver. Intraoperative venography identified a better preserved portal system on the left, and standard MLPVB resulted in excellent shunt flow. The shunt remains patent and the patient asymptomatic 9 months after her operation. Abnormal portal anatomy is not a contraindication to MLPVB.
    Journal of Pediatric Surgery 04/2011; 46(4):749-52. · 1.38 Impact Factor
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    ABSTRACT: Congenital portosystemic shunts (PSS) with preserved intrahepatic portal flow (type II) present with a range of clinical signs. The indications for and benefits of repair of PSS remain incompletely understood. A more comprehensive classification may also benefit comparative analyses from different institutions. All children treated at our institution for type II congenital PSS from 1999 through 2009 were reviewed for presentation, treatment, and outcome. Ten children (7 boys) with type II PSS were identified at a median age of 5.5 years. Hyperammonemia with varying degrees of neurocognitive dysfunction occurred in 80%. The shunt arose from a branch of the portal vein (type IIa; n = 2), from the main portal vein (type IIb; n = 7), or from a splenic or mesenteric vein (type IIc; n = 1). Management included operative ligation (n = 6), endovascular occlusion (n = 3), or a combined approach (n = 1). Shunt occlusion was successful in all cases. Serum ammonia decreased from 130 ± 115 μmol/L preoperatively to 31 ± 15 μmol/L postoperatively (P = .03). Additional benefits included resolution of neurocognitive dysfunction (n = 3), liver nodules (n = 1), and vaginal bleeding (n = 1). Correction of type II PSS relieves a wide array of symptoms. Surgery is indicated for patients with clinically significant shunting. A refined classification system will permit future comparison of patients with similar physiology.
    Journal of Pediatric Surgery 02/2011; 46(2):308-14. · 1.38 Impact Factor
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    ABSTRACT: Biliary atresia is a rare but devastating disease for which hepatoportoenterostomy remains the primary intervention. Increased age at the time of hepatoportoenterostomy is associated with unfavorable outcomes. In this study, we examined trends in age at the time of hepatoportoenterostomy and explored hospital and patient factors associated with more timely diagnosis and treatment. Median ages of patients undergoing hepatoportoenterostomy for biliary atresia were compared using the Kids' Inpatients Database from 1997, 2000, 2003, and 2006. The patient and hospital factors associated with later treatment were compared. Of 192 patients, 13.5% had surgery in 1997, 13.5% in 2000, 36.5% in 2003, and 36.5% in 2006. The overall median age was 65.5 days; the median age was 64 days in 1997, 57.5 days in 2000, 69 days in 2003, and 64 days in 2006 (P = .80). Overall, 71% of patients were treated at nonchildren's hospitals, and although the proportion has increased over time, the trend did not reach significance (P = .12). Hispanic and African American patients were more likely to undergo hepatoportoenterostomy after 60 days of life compared with white patients (Hispanic patients: odds ratio, 3.6; 95% confidence interval, 1.1-12.5; P = .04; African American patients: odds ratio, 2.2; 95% confidence interval, 0.8-6.3; P = .14). Compared with specialized children's centers, treatment at nonchildren's hospitals was associated with delayed hepatoportoenterostomy (odds ratio, 3.5; 95% confidence interval, 1.2-9.8; P = .02). Although early hepatoportoenterostomy is associated with improved outcomes for children with biliary atresia, our study shows the median age at surgery has not significantly changed over 2 decades. Both hospital and socioeconomic factors play a role in the early treatment of biliary atresia.
    Surgery 10/2010; 148(4):785-91; discussion 791-2. · 3.37 Impact Factor

Publication Stats

2k Citations
477.32 Total Impact Points

Institutions

  • 2004–2012
    • Northwestern University
      • Department of Surgery
      Evanston, IL, United States
  • 1999–2011
    • Children's Memorial Hospital
      Chicago, Illinois, United States
  • 2010
    • American College of Surgeons
      Chicago, Illinois, United States
  • 2003–2005
    • Northwestern Memorial Hospital
      • Department of Surgery
      Chicago, Illinois, United States
  • 2000
    • Children's Memorial Medical Center
      Chicago, Illinois, United States
  • 1990–1998
    • University of Toronto
      • • Hospital for Sick Children
      • • Division of General Surgery
      • • Department of Surgery
      Toronto, Ontario, Canada
  • 1988–1995
    • SickKids
      • Department of Surgery
      Toronto, Ontario, Canada