Jacob C Langer

University of Toronto, Toronto, Ontario, Canada

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Publications (303)731.68 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: Background: The management of congenital cystic lung lesions is controversial. Arguments for routine resection during infancy include the possibility of the lesion being Type I pleuropulmonary blastoma (PPB) rather than a cystic congenital pulmonary airway malformation (CPAM). We aimed to identify clinical and radiological features that might distinguish between CPAM and PPB and to develop a diagnostic algorithm based on these features. Methods: All recorded cases of Type I PPB were retrieved from the International PPB Registry and compared with an institutional cohort of children undergoing resection of CPAM (2002-2013) that was noted at some stage to be at least partially cystic. Regression models were created to identify variables that might differentiate CPAM from PPB. Odds ratio (OR) and positive predictive value (PPV) were calculated for each variable and a decision algorithm developed. Results: In 112 cases of Type I PPB and 103 of CPAM, factors favoring a diagnosis of CPAM included prenatal detection (OR 89.4), systemic feeding vessel (OR 61.7), asymptomatic (OR 8.0), and hyperinflated lung (OR 6.6). Factors favoring a diagnosis of PPB included bilateral or multisegment involvement (OR 2.4). A decision algorithm that helps to identify lesions requiring resection and those which can be safely observed is presented. Conclusion: Clinical and radiological features can help to differentiate between CPAM and PPB. Our algorithm allows identification of children at higher risk of PPB in whom we would recommend resection and those at low risk in whom continued close observation is safe.
    Journal of Pediatric Surgery 11/2015; DOI:10.1016/j.jpedsurg.2015.10.019 · 1.39 Impact Factor
  • Jacob C. Langer · Jennifer S. Gordon · Li Ern Chen ·
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    ABSTRACT: Purpose: The purpose of this study was to assess the current status of subspecialization in North American pediatric surgical practices and to evaluate factors associated with subspecialization. Methods: A survey was sent to each pediatric surgical practice in the United States and Canada. For each of 44 operation types, ranging in complexity and volume, the respondents chose one of the following responses: 1. everyone does the operation; 2. group policy - only some surgeons do the operation; 3. group policy - anyone can do it but mentorship required; 4. only some do it due to referral patterns; 5. no one in the group does it. Association of various factors with degree of subspecialization was analyzed using nonparametric statistics with p<0.05 considered significant. Results: Response rate was 70%. There was significant variability in subspecialization among groups. Factors found to be significantly associated with increased subspecialization included free-standing children's hospitals, pediatric surgery training programs, higher number of surgeons, higher case volume, and greater volume of tertiary/quaternary cases. Conclusions: There is wide variation in the degree of subspecialization among North American pediatric surgery practices. These data will help to inform ongoing debate around strategies that may be useful in optimizing pediatric surgical care and patient outcomes in the future.
    Journal of Pediatric Surgery 10/2015; DOI:10.1016/j.jpedsurg.2015.10.038 · 1.39 Impact Factor
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    ABSTRACT: Purpose: The purpose of this study was to define the hematologic response to total splenectomy (TS) or partial splenectomy (PS) in children with hereditary spherocytosis (HS) or sickle cell disease (SCD). Methods: The Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium registry collected hematologic outcomes of children with CHA undergoing TS or PS to 1year after surgery. Using random effects mixed modeling, we evaluated the association of operative type with change in hemoglobin, reticulocyte counts, and bilirubin. We also compared laparoscopic to open splenectomy. Results: The analysis included 130 children, with 62.3% (n=81) undergoing TS. For children with HS, all hematologic measures improved after TS, including a 4.1g/dl increase in hemoglobin. Hematologic parameters also improved after PS, although the response was less robust (hemoglobin increase 2.4g/dl, p<0.001). For children with SCD, there was no change in hemoglobin. Laparoscopy was not associated with differences in hematologic outcomes compared to open. TS and laparoscopy were associated with shorter length of stay. Conclusion: Children with HS have an excellent hematologic response after TS or PS, although the hematologic response is more robust following TS. Children with SCD have smaller changes in their hematologic parameters. These data offer guidance to families and clinicians considering TS or PS.
    Journal of Pediatric Surgery 10/2015; DOI:10.1016/j.jpedsurg.2015.10.028 · 1.39 Impact Factor
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    ABSTRACT: Purpose: Hirschsprung disease (HD) is diagnosed with rectal biopsy. At our institution two services perform these biopsies: pediatric surgery and gastroenterology. Our objective was to review our institutional experience with rectal biopsies to diagnose HD and compare patients and outcomes between the two services. Methods: We reviewed all children undergoing a rectal biopsy for the evaluation of HD at our institution over a 10-year period. Comparisons were made using multiple logistic regression models. Results: We identified 518 children who underwent rectal biopsy for evaluation of HD; 451/518 (87%) were adequate and 56/518 (11%) were positive for HD. A positive biopsy was more likely with delayed passage of meconium (p<0.001), obstructive symptoms (p<0.001), trisomy 21 (p<0.001), full-term gestation (p=0.03), and male gender (p=0.02). Pediatric surgeons biopsied younger patients with more classic symptoms for HD compared to gastroenterologists. Pediatric surgeons were more likely to take adequate (OR 6.0, 95% CI 2.9-12.4, p<0.001) and positive biopsies (OR 6.7 95% CI 2.1-21.2, p=0.001) compared to gastroenterologists. Conclusion: Infants with classic symptoms can reliably be diagnosed with HD by a pediatric surgeon. The work up for HD in older children with constipation should be a collaborative effort between pediatric surgery and gastroenterology.
    Journal of Pediatric Surgery 10/2015; DOI:10.1016/j.jpedsurg.2015.10.047 · 1.39 Impact Factor

  • Ultrasound in Obstetrics and Gynecology 09/2015; 46(S1):79-79. DOI:10.1002/uog.15185 · 3.85 Impact Factor
  • Nigel J Hall · Priscilla P L Chiu · Jacob C Langer ·
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    ABSTRACT: The optimal management of prenatally diagnosed asymptomatic congenital pulmonary airway malformations (CPAM) is controversial. Since there is a paucity of data relating to surgical risks in this specific population, we reviewed our experience to further inform this controversy. Ethically approved 10 year (2004-2013) retrospective review. Patients were included only if the CPAM was diagnosed prenatally and remained asymptomatic. Indication for surgery was physician recommendation and/or parental choice. Sixty patients were identified. Median age at surgery was 6.5 months (range 65 days to 9.6 years). Resections were performed thoracoscopically (n = 51, one conversion) or by thoracotomy (n = 9). Surgical time was 2.5 hr (43 min to 4.75 hr). A chest drain was used in 58/60 and remained in situ 53 hr (23-108). There were no intra-operative complications or blood transfusions. All patients were extubated at the end of the procedure with no re-intubations. Post-operative hospitalization was 73.4 hr (23.8 hr to 4.2 days). Overall, complications occurred in 14/60 (23%). Eleven were minor but three were major: tension pneumothorax associated with new presentation of a small previously undiagnosed diaphragmatic hernia 5 days following resection; aggressive fibromatosis of the chest wall in the region close to resection 2 years later; and near-fatal hypovolemic cardiac arrest due to massive haemorrhage from a feeding vessel on postoperative day 7. There were no deaths and no cases of pleuropulmonary blastoma. Resection of prenatally diagnosed asymptomatic CPAM is associated with a significant risk of complications, which may be life threatening. These data contribute to a balanced discussion of risks and benefits for these children. Pediatr Pulmonol. 2015; 9999:1-6. © 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc.
    Pediatric Pulmonology 07/2015; DOI:10.1002/ppul.23255 · 2.70 Impact Factor

  • Annals of surgery 06/2015; DOI:10.1097/SLA.0000000000001284 · 8.33 Impact Factor
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    ABSTRACT: Pancreatitis-Panniculitis-Polyarthritis (PPP) syndrome is rare and its physiopathology unclear. A 6-year old boy suffered traumatic pancreatitis complicated by PPP syndrome. Extensive investigations demonstrated high levels of pancreatic lipase and fatty acids in the affected peripheral tissues. These findings support the sequence of peripheral lipolysis and fatty acid accumulation inducing tissue inflammation.
    Journal of Pediatric Surgery Case Reports 04/2015; 33(5). DOI:10.1016/j.epsc.2015.03.014
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    ABSTRACT: The primary objective was to determine the diagnostic accuracy of a serial ultrasound (US) clinical diagnostic pathway to detect appendicitis in children presenting to the emergency department (ED). The secondary objective was to examine the diagnostic performance of the initial and interval US and to compare the accuracy of the pathway to that of the initial US. This was a prospective cohort study of 294 previously healthy children 4 to 17 years old with suspected appendicitis and baseline pediatric appendicitis scores of ≥2, who were managed with the serial US clinical diagnostic pathway. This pathway consisted of an initial US followed by a clinical reassessment in each patient and an interval US and surgical consultation in patients with equivocal initial US and persistent concern about appendicitis. The USs were interpreted by published criteria as positive, negative, or equivocal for appendicitis. Children in whom this pathway did not rule in or rule out appendicitis underwent computed tomography (CT). Cases with missed appendicitis, negative operations, and CTs after the pathway were considered inaccurate. The primary outcome was the diagnostic accuracy of the serial US clinical diagnostic pathway. The secondary outcomes included the test performance of the initial and interval US imaging studies. Of the 294 study children, 111 (38%) had appendicitis. Using the serial US clinical diagnostic pathway, 274 of 294 children (93%, 95% confidence interval [CI] = 90% to 96%) had diagnostically accurate results: 108 of the 111 (97%) appendicitis cases were successfully identified by the pathway without CT scans (two missed and one CT), and 166 of the 183 (91%) negative cases were ruled out without CT scans (14 negative operations and three CTs). The sensitivity of this pathway was 108 of 111 (97%, 95% CI = 94% to 100%), specificity 166 of 183 (91%, 95% CI = 87% to 95%), positive predictive value 108 of 125 (86%; 95% CI = 79% to 92%), and negative predictive value 166 of 169 (98%, 95% CI = 96% to 100%). The diagnostic accuracy of the pathway was higher than that of the initial US alone (274 of 294 vs. 160 of 294; p < 0.0001). Of 123 patients with equivocal initial US, concern about appendicitis subsided on clinical reassessment in 73 (no surgery and no missed appendicitis). Of 50 children with persistent symptoms, 40 underwent interval US and 10 had surgical consultation alone. The interval US confirmed or ruled out appendicitis in 22 of 40 children (55.0%) with equivocal initial US, with one false-positive interval US. The serial US clinical diagnostic pathway in suspected appendicitis has an acceptable diagnostic accuracy that is significantly higher than that of the initial US and results in few CT scans. This approach appears most useful in children with equivocal initial US, in whom the majority of negative cases were identified at clinical reassessment and appendicitis was diagnosed by interval US or surgical consultation in most study patients. © 2015 by the Society for Academic Emergency Medicine.
    Academic Emergency Medicine 03/2015; 22(4). DOI:10.1111/acem.12631 · 2.01 Impact Factor
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    ABSTRACT: Neonates with cholestasis may undergo many tests before biliary atresia (BA) or an alternative diagnosis is reached, and delayed intervention may worsen outcomes. An optimal diagnostic approach to reduce risk, cost, and delay has yet to be defined. The purpose of this study was to develop an algorithm that rapidly and accurately excludes BA for infants with cholestatic jaundice. A single-center retrospective comparison of diagnostic workup was made between cholestatic infants with BA, and those without BA who underwent hepatobiliary iminodiacetic acid (HIDA) scan during admission. Patients were born between 2000 and 2010 and those older than 100days at assessment were excluded. Sensitivity and specificity analysis of predictive variables was performed and an algorithm constructed. There were 45 BA and 167 non-BA patients. Some variables were 100% sensitive for the exclusion of BA: conjugated bilirubin <2.5mg/dL, gamma-glutamyl transpeptidase <150U/L, excretion on HIDA, or a normal percutaneous cholangiogram. Clinical variables and ultrasound were less useful as screening tests owing to low specificity and sensitivity, respectively. Liver biopsy was 98% sensitive and 84% specific in the diagnosis of BA. An algorithm was constructed that rules out BA with a negative laparotomy rate of 3-22%. We propose a screening algorithm for infants with conjugated hyperbilirubinemia that permits efficient exclusion of BA with minimal invasive testing and with a low risk of negative laparotomy. This algorithm now requires prospective evaluation to determine its diagnostic accuracy and its ability to reduce hospital costs, patient morbidity, and time to Kasai portoenterostomy in patients with BA. Copyright © 2015 Elsevier Inc. All rights reserved.
    Journal of Pediatric Surgery 03/2015; 50(3):363-70. DOI:10.1016/j.jpedsurg.2014.08.014 · 1.39 Impact Factor
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    Jacob C. Langer · Paul W. Wales ·

    Journal of Pediatric Surgery 03/2015; 50(5). DOI:10.1016/j.jpedsurg.2015.02.019 · 1.39 Impact Factor

  • Journal of Pediatric Surgery 03/2015; 50(3):497-8. DOI:10.1016/j.jpedsurg.2014.12.003 · 1.39 Impact Factor
  • Paola Luca · Elizabeth Dettmer · J.C. Langer · J.K. Hamilton ·
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    ABSTRACT: Bariatric surgery has been increasingly performed in adolescents over the past decade. Consensus guidelines have been developed to help heath care teams select adolescent candidates for surgery. Reports of short-term outcomes in adolescents have demonstrated similar BMI reduction and safety as in the adult population. There are several issues specific to adolescents that require further consideration, including a lower age limit and BMI at surgery, the optimal choice of bariatric procedure, the potential for the development of disordered eating and weight recidivism after surgery, and the extent of psychological and developmental assessment prior to performing these procedures. With the ongoing increase in the number of adolescent bariatric surgeries performed, it will be essential for high-level evidence with long-term follow-up to be generated to help address these issues and guide health care teams caring for teens with obesity.
    Pediatric and Adolescent Medicine 01/2015; 19:179-186. DOI:10.1159/000368135
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    ABSTRACT: The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005-2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short-term adverse events (AEs) (≤ 30 days after surgery), and long-term AEs (31-365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1 ± 1.8 gm/dl, 52 week 12.8 ± 1.6 gm/dl; mean ± SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short-term AEs and 11% rate of long-term AEs. As we accumulate more subjects and longer follow-up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision-making process.
    American Journal of Hematology 11/2014; 90(3). DOI:10.1002/ajh.23888 · 3.80 Impact Factor
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    ABSTRACT: Purpose: Children with perianal Crohn's disease (PCD) are a unique and diverse patient population. The purpose of this study was to describe the spectrum of disease and role of surgery. Methods: A retrospective chart review of all children having at least one surgical intervention for PCD over 10 years was performed. Results: Fifty-seven patients (63% male) aged 0.5-17 (median 13) years were identified. Perianal disease consisted of skin tags (49%), superficial fistulae (49%), deep fistulae (37%), superficial abscesses (68%), deep abscesses (9%), skin breakdown (19%), and anal strictures (7%). 84% received anti-TNF therapy, with 27% treated with a second anti-TNF medication. Minor surgical procedures, commonly done during anti-TNF therapy, included abscess drainage (67%) and seton placement (33%). Major surgical procedures, done almost exclusively after anti-TNF failure, included defunctioning ileostomy (23%) and subtotal colectomy (9%). Follow-up ranged from 7 to 160 (median 54) months. Conclusions: Pediatric PCD has a wide range of disease severity. Minor surgery provides adequate drainage before and during anti-TNF therapy, while major surgery plays a role in medically refractory disease. Appropriate surgical intervention remains an important part of the treatment paradigm.
    Journal of Pediatric Surgery 11/2014; 50(1). DOI:10.1016/j.jpedsurg.2014.10.034 · 1.39 Impact Factor
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    Evan J. Propst · Simon C. Ling · Alan Daneman · Jacob C. Langer ·
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    ABSTRACT: Laryngoscope, 124:2182–2185, 2014
    The Laryngoscope 09/2014; 124(9). DOI:10.1002/lary.24650 · 2.14 Impact Factor
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    ABSTRACT: Background: Despite randomized controlled trials and meta-analyses, it remains unclear whether laparoscopic pyloromyotomy (LP) carries a higher risk of incomplete pyloromyotomy and mucosal perforation compared with open pyloromyotomy (OP). Methods: Multicenter study of all pyloromyotomies (May 2007-December 2010) at nine high-volume institutions. The effect of laparoscopy on the procedure-related complications of incomplete pyloromyotomy and mucosal perforation was determined using binomial logistic regression adjusting for differences among centers. Results: Data relating to 2830 pyloromyotomies (1802 [64%] LP) were analyzed. There were 24 cases of incomplete pyloromyotomy; 3 in the open group (0.29%) and 21 in the laparoscopic group (1.16%). There were 18 cases of mucosal perforation; 3 in the open group (0.29%) and 15 in the laparoscopic group (0.83%). The regression model demonstrated that LP was a marginally significant predictor of incomplete pyloromyotomy (adjusted difference 0.87% [95% CI 0.006-4.083]; P=0.046) but not of mucosal perforation (adjusted difference 0.56% [95% CI -0.096 to 3.365]; P=0.153). Trainees performed a similar proportion of each procedure (laparoscopic 82.6% vs. open 80.3%; P=0.2) and grade of primary operator did not affect the rate of either complication. Conclusions: This is one of the largest series of pyloromyotomy ever reported. Although laparoscopy is associated with a statistically significant increase in the risk of incomplete pyloromyotomy, the effect size is small and of questionable clinical relevance. Both OP and LP are associated with low rates of mucosal perforation and incomplete pyloromyotomy in specialist centers, whether trainee or consultant surgeons perform the procedure.
    Journal of Pediatric Surgery 07/2014; 49(7):1083-6. DOI:10.1016/j.jpedsurg.2013.10.014 · 1.39 Impact Factor
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    ABSTRACT: Large solid sacrococcygeal teratomas (SCT) can cause high output cardiac failure and fetal or neonatal death. We describe the outcomes of minimally invasive antenatal procedures for the treatment of fetal SCT. Material and methodsCase review of 5 fetuses with large SCT's treated antenatally using minimally invasive techniques and systematic literature review on fetal therapy for solid SCT's. Five women were referred between 17.7-26.6 weeks gestation for large fetal SCT's with evidence of fetal cardiac failure. Vascular flow to the tumors was interrupted by fetoscopic laser ablation (n = 1), radiofrequency ablation (RFA, n = 2) and interstitial laser ablation +/- vascular coiling(n = 2). There were 2 intra-uterine fetal deaths. The other 3 cases resulted in preterm labor within 10 days of surgery. One neonate died. Two survived without procedure related complications but had long-term morbidity related to prematurity. Systematic literature review revealed 15 SCT's treated minimally invasively for (early) hydrops. Including ours, 6 of 20 hydropic fetuses survived after minimally invasive therapy (30%). Survival after RFA or interstitial laser was 45% (n = 5/11). Of 12 fetuses treated for SCT without obvious hydrops, 67% (n = 8/12) survived. Mean gestational age at delivery after minimally invasive therapy was 29.7 ± 4.0 weeks. Survival after open fetal surgery in hydropic fetuses was 55% (n = 6/11) with a mean gestational age at delivery of 29.8 ± 2.9 weeks. Fetal therapy can potentially improve perinatal outcomes for hydropic fetuses with solid SCT's but is often complicated by intrauterine death and preterm birth.
    Ultrasound in Obstetrics and Gynecology 06/2014; 43(6). DOI:10.1002/uog.13315 · 3.85 Impact Factor

  • Annals of Surgery 05/2014; 261(1). DOI:10.1097/SLA.0000000000000748 · 8.33 Impact Factor
  • Nada Gawad · Dafydd A. Davies · Jacob C. Langer ·
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    ABSTRACT: Background Longer wait time for infant inguinal hernia (IH) repair is associated with higher complication rates. We wished to determine if socioeconomic and demographic factors influence wait times for IH repair. Methods Children < 2 yo with IH at a Canadian children’s hospital were retrospectively reviewed. Days from diagnosis to surgical consultation (W1) and from consultation to repair (W2) were collected along with demographic, medical ,and socioeconomic data. Linear regression analysis was performed. Results 131 patients were appropriate for analysis (82.4% male). Median distance to hospital was 27.5 km (IQR = 10.5-50.4) and median income was $34,477 (IQR = 30,127-41,986). Median W1, W2. and Wtotal (W1 + W2) were 24 (IQR = 8-48), 43 (IQR = 21-69) and 79 (IQR = 38-112) days, respectively. Wait times were shorter in infants who were male (p = 0.044), symptomatic (p < 0.001), diagnosed in the ED (p < 0.001), or had an incarcerated hernia (p = 0.006). They were longer for premature infants (p = 0.009) and those with significant comorbidities (p = 0.018). Neither income (p = 0.328) nor distance from hospital (p = 0.292) was associated with longer wait times. Conclusion Wait times for IH repair were appropriately influenced by medical risk factors. Income and distance to hospital did not appear to influence wait times. A population-based study is needed to determine if these findings reflect a general trend within the Canadian health care system.
    Journal of Pediatric Surgery 05/2014; 49(5). DOI:10.1016/j.jpedsurg.2014.02.064 · 1.39 Impact Factor

Publication Stats

7k Citations
731.68 Total Impact Points


  • 1990-2015
    • University of Toronto
      • • Department of Surgery
      • • Hospital for Sick Children
      • • Department of Obstetrics and Gynaecology
      • • Division of General Surgery
      • • Division of Neonatology
      Toronto, Ontario, Canada
  • 1987-2015
    • SickKids
      • • Department of Surgery
      • • Division of General Surgery
      Toronto, Ontario, Canada
  • 2011
    • Institute for Clinical Evaluative Sciences
      Toronto, Ontario, Canada
    • University of Texas Southwestern Medical Center
      • Department of Pediatrics
      Dallas, Texas, United States
  • 2009
    • Child & Family Research Institute
      Vancouver, British Columbia, Canada
  • 1992-2009
    • McMaster University
      • Department of Surgery
      Hamilton, Ontario, Canada
  • 2008
    • Cincinnati Children's Hospital Medical Center
      • Division of Pediatric General and Thoracic Surgery
      Cincinnati, OH, United States
  • 1996-2001
    • Washington University in St. Louis
      • Department of Surgery
      San Luis, Missouri, United States
  • 1999
    • Saint Louis Zoo
      San Luis, Missouri, United States
  • 1996-1998
    • St. Luke's Hospital (MO, USA)
      Saint Louis, Michigan, United States
  • 1993
    • University of Missouri - St. Louis
      Saint Louis, Michigan, United States
  • 1989
    • The University of Manchester
      Manchester, England, United Kingdom
  • 1987-1989
    • University of California, San Francisco
      • • Department of Surgery
      • • Division of Pediatric Surgery
      San Francisco, CA, United States