NASPGHAN Single Topic Symposium
Polyps Matter:  Pediatric Polyposis and GI Cancer Syndromes
Wednesday, October 4, 2023

Course Director
Thomas M. Attard, MD
Children's Mercy Hospital, Kansas City

Planning Committee
Steven H. Erdman, MD, Nationwide Children's Hospital
Claudia Phen, MD, UTSW/Children's Health
Isabel Rojas, MD, UT Southwestern
Jacob Kurowski, MD, Cleveland Clinic
Suzanne P. MacFarland, MD, Children's Hospital of Philadelphia
Paul Tran, MD, Phoenix Children's Hospital
Sabina Ahmed Mir, MD, University of North Carolina Chapel Hill
Mary Zachos, MD, McMaster University

8:00 AM - 8:15 AM Polyps Matter: Pediatric polyposis and GI cancer syndromes
8:00 AM          Welcome and Overview
                   Thomas M. Attard, MD, Children's Mercy Hospital, Kansas City
8:05 AM          Patient vignette

8:15 AM - 9:35 AM General Session I
Polyposis . . . what you need to know
Moderators: Steven H. Erdman, MD, Nationwide Children's Hospital and Sabina Ahmed Mir, MD, University of North Carolina Chapel Hill

8:15 AM        Juvenile polyps: Numbers matter (or do they?)
                   Victor L. Fox, MD, Boston Children's Hospital
                   Learning objectives:

• Recognize clinical characteristics that differentiate sporadic juvenile polyps from hereditary juvenile polyps
• Understand the “grey zone” for polyp number and histology
• Review evidence for endoscopic surveillance

8:35 AM        Familial adenomatous polyposis: Preparing for patient care
                   Warren Hyer, MB ChB MRCP FRCPCH, St Marks Polyposis Registry, St Marks Hospital, London UK
                   Learning objectives:

• Identify the key clinical characteristics of FAP relating to the disease, specific mutation, genetics , family history and extra colonic manifestations
• Describe the necessary management plan including a the role of the multidisciplinary approach; and the role, method and timing  of endoscopic surveillance
• Identify endoscopic findings and threshold/timing that warrant referral to surgery

9:10 AM        Peutz Jeghers Syndrome: Freckles are never good
                   Wenly Ruan, MD, Baylor College of Medicine
                   Learning objectives:

• Discuss overview and epidemiology of Peutz Jeghers syndrome
• Discuss current management strategies of Peutz Jeghers syndrome
• Discuss timing of endoscopy
• Endoscopic management strategies for management of small bowel polyps

9:25 AM       Discussion/Q&A

9:35 AM - 9:45 AM  Break 

9:45 AM - 10:40 AM Keynote Session
Moderator: Carol Durno, MD, Hospital for Sick Children

9:45 AM        The GI Polyposis Syndromes: Mechanisms and implications
                   C Richard Boland, MD, UCSD School of Medicine
                   Learning Objectives: The lecture will explain:

• The nature of the polyposis associated with: familial adenomatous polyposis, Lynch syndrome, Peutz-Jeghers syndrome, juvenile polyposis syndrome and Cowden’s syndrome
• The pathobiological mechanisms associated with the development of the polyps – and cancers - in each syndrome
• The clinical implications of these different biological mechanisms

10:40 AM - 11:45 AM General Session II
Polyposis: Recognition, risks and surveillance strategies
Moderators: Catharine Walsh, MD, MEd, PhD, FRCPC, Hospital for Sick Children and Christine Drogan, MS, LGCG, University of Chicago

10:40 AM       Mismatch repair: Not just in adults
                   Carol Durno, MD, Hospital for Sick Children
                   Learning objectives:

• Recognize Lynch Syndrome in young people
• Understand what we know about CMMRD in 2023
• Discuss surveillance strategies in CMMRD

11:05 AM      Panel 
                   Unusual polyps and Rare Polyposis Syndromes:  Juvenile Polyposis of Infancy (JPI)
                   Claudia Phen, MD, UTSW/Children's Health
                   Learning objectives:

• Overview of clinical features and natural history of JPI
• Discuss management strategies with endoscopic polyp debulking, colectomy, and mTOR inhibition

                   Unusual polyps and Rare Polyposis Syndromes: PTEN
                   Jacob Kurowski, MD, Cleveland Clinic
                   Learning objectives:

• Review when to test for PTEN Hamartoma Tumor Syndrome.
• Identify common GI manifestations and screening recommendations.

                   Unusual polyps and Rare Polyposis Syndromes: Serrated Polyps
                   Monique Barakat, MD, PhD, Stanford University School of Medicine, Lucille Packard Children's Hospital
                   Learning objectives:

• Overview of clinical features and natural history of serrated polyps
• Discuss serrated polyp associated syndromes
• Discuss endoscopic and overall management of serrated polyps

11:35 AM      Discussion/Q&A

11:45 AM - 12:35 PM Lunch and Concurrent Session I 
Polyposis Syndromes: Endoscopic and Other Clinical Management Dilemmas
Group Discussion
Panellists:
             Monique Barakat, MD, PhD, Stanford University School of Medicine, Lucille Packard Children's Hospital
             Warren Hyer, MBChB, MRCP, FRCPCH, St Marks Polyposis Registry, St Marks Hospital, London UK
         David Vitale, MD, Cincinnati Children’s
Moderators:
             Diana Lerner, MD, Medical College of Wisconsin
             Claudia Phen, MD, UTSW/Children's Health
Learning objectives:

• Presentation of 3 challenging patient cases by Dr. Hyer and Dr. Vitale
• Group discussion regarding endoscopic, medical, and surgical management strategies

Polyposis Syndromes: The Role of Genetic Counselling in Management
Moderators: Suzanne P. MacFarland, MD, Children's Hospital of Philadelphia and Melyssa Aronson, MS (C)CGC, Sanai Health
Panel:
            Christine Drogan, MS, LGCG, University of Chicago
            Kristin Zelley, MS, Children's Hospital of Philadelphia
            Learning objectives:

• Define the role of a genetic counselor in the evaluation and management of pediatric patients with polyposis syndromes
• Evaluate the benefits of genetic counseling services for families of patients with polyposis syndromes
• Examine how a genetic diagnosis can influence extra-intestinal management for polyposis patients

12:35 PM - 2:05 PM General Session III
Polyposis syndrome: A holistic approach
Moderators: Jacob Kurowski, MD, Cleveland Clinic and Colleen Flahive, MD, Nationwide Children’s Hospital

12:35 PM      Beyond polyp removal: Responding to the psychological needs of pediatric patients diagnosed with polyposis syndromes 
                   Christina Low Kapalu, PhD, Children's Mercy
                   Learning objectives: By the end of this training, learners will be able to:

• Discuss what is known about the psychosocial impacts of pediatric polyposis syndromes
• Identify medical traumatic stress reactions in patients and families
• Apply three trauma informed care principles to clinical practice
• Evaluate the impact of multigenerational medical trauma on decision-making

1:15 PM        Genetic Testing in Polyposis Families: What is available and what could result?
                   Melyssa Aronson, MS (C)CGC, Mount Sinai Hospital
                   Learning objectives:

• Discuss genetic testing panels to investigate polyposis
• Understand key differences in types of genetic testing results, such as de novo, mosaicism and no pathogenic variant identified

1:40 PM       Disparities matter: Understanding the impact of social determinants of health for patients with polyposis syndromes
                   Monica M Alvarado, MS, CGC, Kaiser Permanente
                   Learning objectives:

• Understand key socioeconomic and demographic challenges in accessing specialized and multidisciplinary care
• Identify potential resources and approaches to help overcome these challenges

1:55 PM       Discussion/Q&A

2:05 PM - 3:05 PM Concurrent Session II 
It takes a village: Developing a multidisciplinary polyposis team
Moderators: Steven H. Erdman, MD, Nationwide Children's Hospital and Mary Zachos, MD, McMaster University

2:05 PM     How to start a polyposis program
                   Edward Hoffenberg, MD, Children's Hospital Colorado
                   Learning objectives: To be able to

• Develop your own approach to starting a polyposis program
• Have confidence in your “ask” strategy
• Build a periodic reassessment into the program

2:20 PM    Panel Discussion: How we do it
                   Suzanne P. MacFarland, MD, Children's Hospital of Philadelphia
                   Kristin Zelley, MS, Children's Hospital of Philadelphia
                   Learning objectives:

• Facilitating collaboration between subspecialties to maximize convenience and support for patients and families
• List benefits to having multiple subspecialties involved in care of pediatric polyposis patients

2:50 PM    Moving on: Transitioning the pediatric polyposis patient
                   Jacob Kurowski, MD, Cleveland Clinic
                   Learning objectives:

• Identify the need for a method of transition to adults for the pediatric patient with polyposis
• Recognize the team members recommended for ongoing care in polyposis
• Define optimal transitional care for pediatric polyposis syndromes

Outside the colon: Extracolonic manifestations of polyposis syndromes
Moderators: Lee Bass, MD, Ann & Robert H Lurie Children's Hospital and David Vitale, MD, Cincinnati Children's Hospital

2:05 PM     Gastric polyps and GAPPS
                   Sabina Ahmed Mir, MD, University of North Carolina Chapel Hill
                   Learning objectives:

• Review the classification of gastric polyp
• Surveillance and management of gastric polyps
• When to suspect GAPPS

2:20 PM     Juvenile Polyposis (JPS)/Hereditary Hemorrhagic Telangiectasia (HTT): an overlap of syndromes
                   Claudia Phen, MD, UTSW/Children's Health
                   Learning objectives:

• Overview of clinical features and diagnostic criteria for HHT
• Genetic testing and SMAD4 mutation
• Screening and surveillance for HHT

2:35 PM    Extra-intestinal tumors of FAP
                   Craig W. Lillehei, MD, Boston Children's Hospital
                   Learning objectives:

• Review range of tumors
• Discuss management options

3:05 PM - 4:50 PM General Session IV
Characterizing and managing polyps: Staying out of trouble
Moderators: Paul Tran, MD, Phoenix Children's Hospital and Monique Barakat, MD, PhD, Stanford University School of Medicine, Lucille Packard Children's Hospital

3:05 PM      Endoscopically managing polyps: Size matters
                   Douglas Fishman, MD, FASGE, Texas Children's Hospital
                   Learning objectives:

• Describe current techniques in pediatric polypectomy
• Discuss adverse events and complications of polyps and their subsequent polypectomy
• Highlight practical applications of removing large polyps

3:25 PM     Characterizing polyps: Appearance matters
                   Thomas M. Attard, MD, Children's Mercy Hospital, Kansas City
                   Learning objectives:

• Identify key morphologic characteristics of gastrointestinal polyps that may affect endoscopic approach
• Review established classification conventions for different gastrointestinal polyps
• Introduce techniques and relevance for sizing polyps

3:45 PM     Surgically managing polyps: Collaboration matters
                   Craig W. Lillehei, MD, Boston Children's Hospital
                   Learning objectives:

• Appreciate surgical options for FAP management
• Discuss factors relevant to timing intervention
• Review surgical outcomes/complications

4:10 PM    Capsule endoscopy and imaging: Visualizing matters
                   Isabel Rojas, MD, UT Southwestern
                   Learning objectives:

• Review evidence for capsule endoscopy vs small bowel imaging in hereditary polyposis syndromes
• Discuss the clinical value and utility of video capsule endoscopy in hereditary polyposis syndromes
• Identify what type of patients with hereditary polyposis syndromes will benefit from small bowel surveillance

4:30 PM    Therapies beyond resection: Do medicines matter?
                   Seth Septer, MD, Children's Hospital Colorado
                   Learning objectives:

• Review the current evidence regarding medications, which may be used for patients with hereditary polyposis syndromes
• Discuss natural supplements or dietary modifications, which may be used for patients with hereditary polyposis syndromes
• Review several of the novel agents, which are being explored for use in patients with hereditary polyposis syndromes

4:45 PM    Discussion/Q&A

4:50 PM - 5:00 PM  General Session V
Closing Remarks
Thomas M. Attard, MD, Children's Mercy Hospital, Kansas City