Skip Navigation LinksHome > January 2013 - Volume 56 - Issue > Congenital Anomalies
Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/01.mpg.0000425837.05154.2d
Congenital Anomalies

Congenital Anomalies

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Congenital Anomalies

Task Force Members

Jacqueline Fridge, Chair

Michael D. Bates

William J. Byrne

Sanjeev Dutta

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Importance of Area

Because many children with congenital anomalies present initially to a pediatric gastroenterologist and usually continue their posttreatment long-term care with a pediatric gastroenterologist, trainees should be familiar with these anomalies and the diagnosis, treatment options, and long-term prognosis for these patients.

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Medical Knowledge

Trainees in pediatric gastroenterology should understand the embryologic origins, normal histology, and vascular supply of the entire digestive system and have a detailed understanding of fundamental developmental processes, including intestinal rotation and fixation. They should know the incidence, presentation, and natural history of congenital anomalies, both treated and untreated. For anomalies having a known genetic basis, they should understand the pattern of inheritance and implications for the family. Trainees should be familiar with the differential diagnosis of the various anomalies and the various treatment approaches, even though many therapy options fall outside the role of a pediatric gastroenterologist. When surgical treatment is needed, trainees should understand the general techniques and potential complications.

Trainees should understand the malformations that can directly affect feeding:

* Pierre Robin sequence

* Cleft lip and palate

* Treacher Collins syndrome

* Anomalies of and around the esophagus (eg, atresia, web, tracheoesophageal fistula [TEF], and vascular ring)

Trainees should thoroughly understand the advantages and disadvantages of feeding methods, how feeding choices can be individualized for each child's needs, and that feeding choices in infancy can have significant consequences for future feeding abilities.

Trainees should be familiar with anatomic anomalies of the foregut (including the liver, biliary system, and pancreas). Trainees should understand the presentation and complications of anatomic variants of hepatic and pancreatic anatomy and vascular supply, including congenital biliary atresia, choledochal cyst, annular pancreas, and pancreas divisum. They should know the strengths and pitfalls of various diagnostic methods (eg, ultrasonography, computerized tomography [CT], magnetic resonance cholangiopancreatography [MRCP], and endoscopic retrograde cholangiopancreatography [ERCP]) in assessing the pancreas and biliary tract.

Trainees should be aware of other foregut anomalies, including anomalies of the stomach and duodenum (eg, web, duplication, atresia), which may present with upper GI tract obstruction.

Trainees should be aware of important congenital anomalies of the midgut, including anomalies of the small intestine (eg, web, duplication, atresia, Meckel diverticulum). They should comprehend abnormalities of rotation and fixation, including malrotation and volvulus. Trainees should know that the differential diagnosis of a small bowel obstruction in the newborn also includes meconium ileus from cystic fibrosis. Trainees should be familiar with anomalies of the hindgut, including abnormalities of fixation, atresias, and anorectal malformations.

Trainees should understand the wide age range of presentation of these and other associated anomalies, and they should be familiar with methods of diagnosing these anomalies and indications for surgery. They should also know diagnostic and therapeutic approaches to upper GI (UGI) and lower GI (LGI) obstruction and be able to recognize the symptoms and signs of bacterial overgrowth that may accompany obstruction or blind loops.

Trainees also should be familiar with the treatment and consequences of abdominal wall defects (eg, gastroschisis, omphalocele) and of congenital diaphragmatic hernias. Trainees should recognize that abdominal wall and diaphragm defects are typically associated with anomalies of intestinal rotation, but that in most cases these are clinically inconsequential.

Trainees should be aware of syndromes and associations that frequently include congenital anomalies of the GI tract (eg, Down syndrome, VACTERL association [a combination of at least 3 of the following defects: vertebral defects, anal atresia, cardiac defects, TEF, renal anomalies, and limb abnormalities]). Trainees should understand congenital anomalies that can cause disorders of defecation (eg, spina bifida, tethered cord, Hirschsprung disease), and they should be familiar with syndromic conditions that have hepatobiliary associations (eg, Alagille syndrome). Trainees should be familiar with the presentation, differential diagnosis, evaluation, and long-term treatment of these disorders.

Trainees should appreciate that many of these congenital anomalies of the intestinal tract have long-term motility consequences because of disordered fetal GI development, and they should become proficient in treatment approaches for the feeding-intolerant child.

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Required Patient Care Experiences/Skills

Trainees should develop the necessary skills and experience in history taking and physical examination of neonates and children of all ages with regard to potential congenital anomalies. They should learn to exercise clinical judgment about which children require further investigation and which tests are most appropriate based on the anomaly being considered and the age of the patient (eg, barium enema, rectal suction biopsy, anorectal manometry to evaluate for Hirschsprung disease).

Trainees’ experience should include time evaluating neonates in a high-risk newborn nursery. They should be able to counsel families about prognosis and management of congenital anomalies in the neonatal period; for example, they should be able to present the prognosis for an infant with omphalocele or gastroschisis, and the factors that predict a favorable outcome.

Trainees should participate in inpatient and outpatient management of the preoperative and postoperative care of patients with anomalies requiring surgery in the neonatal period (eg, esophageal atresia). Trainees should be able to determine the appropriate uses of various feeding devices and should develop skills in handling commonly used equipment and solving potential complications. This experience should include placement, care, and troubleshooting gastrostomy and jejunostomy tubes and other feeding devices.

Trainees should develop skills in the procedures used to assess and/or manage children with congenital anomalies (eg, percutaneous gastrostomy tube placement in the neonate with Pierre Robin sequence who cannot feed orally). Trainees should be able to perform rectal suction biopsies to evaluate for Hirschsprung disease and interpret the histopathologic findings. They should understand the implications of congenital malformations and their prior surgical repair when considering endoscopic evaluation. They should be familiar with the indications, technique, and complications of dilatations (eg, of an esophageal stricture following repair of esophageal atresia).

Trainees should develop skills in interpreting the results of investigations used to assess for congenital anomalies, including diagnostic imaging techniques:

* Ultrasonography

* Fluoroscopy

* CT

* Magnetic resonance imaging (MRI)

Trainees should be able to participate with competence in multidisciplinary teams, including working closely with pediatric surgeons, geneticists, neonatologists, dietitians, and feeding therapists. They should be skilled in communicating effectively with nursing staff and familiar with the home supply needs of children with feeding or continence issues, and how to provide for those needs. They also should be familiar with specific enteral and parenteral nutrition options for maintaining appropriate nutrition in these patients.

Trainees should have the opportunity to maintain continuity with their patients during the course of their training and to experience the long-term care of patients with congenital anomalies and how these will or will not affect the patients’ ability to achieve developmental milestones (eg, ability to achieve continence of stool for a child born with imperforate anus).

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Examples of Relevant Competencies—Congenital Anomalies
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Functional GI and Motility Disorders

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Task Force Members

Gisela G. Chelimsky, Chair

Carlo DiLorenzo

Samuel Nurko

Manu Raj Sood

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Importance of Area

Because functional GI disorders (FGID) and motility disorders are common in children, trainees in pediatric gastroenterology require comprehensive exposure to the diagnosis and treatment of these disorders and their complications, as well as a thorough understanding of their pathophysiology.

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Medical Knowledge

Because of the wide range of disorders in this category, the discussion of medical knowledge is divided into 2 sections: FGID and disorders of transit.

FGID: Trainees in pediatric gastroenterology should understand the types and current classification of FGID by the Rome III criteria, including the disorders in the 2 main groups: neonates and infants and children and adolescents (Table 1). They should be familiar with the epidemiology of FGID, including the following:

* Prevalence in different geographic areas

* Age-related vulnerability

* Sex predominance of certain disorders

* Natural history

Trainees should thoroughly comprehend the biopsychosocial model of illness and the concept of the brain–gut axis in the evaluation and treatment of FGID. They also should be cognizant of fictitious disorder by proxy and how it may mimic organic or functional disease.

For each FGID syndrome (Table 1), trainees should know the following:

* The diagnostic criteria

* The alarm signs that should prompt further evaluation

* The role of different diagnostic tests (including indications and limitations of manometry testing in FGID)

* The indications and adverse effects of the available medications

* The role of psychological evaluation and behavioral modifications as part of the multidisciplinary approach to children with FGID

TABLE 1. Rome III classification of FGID

Disorders of Transit: Trainees should comprehend the swallowing mechanism, including the role of the central nervous system in swallowing. They should be familiar with the indications and technique of performing tests to evaluate swallowing disorders in children.

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Trainees should know the anatomy and innervation of the different portions of the esophagus and understand the most common causes of esophageal dysmotility, including those occurring in eosinophilic esophagitis and other kinds of esophagitis and in achalasia. They should have sufficient exposure to children with a history of TEF to recognize the dysmotility associated with this entity and be able to manage symptomatic children with TEF.

Trainees should be familiar with the pathophysiology and modes of presentation of gastroesophageal reflux disease (GERD) and how it is distinguished from physiologic gastroesophageal reflux. They should understand the differential diagnosis of GERD, its evaluation (including pH monitoring, esophageal impedance monitoring, and endoscopy with biopsies), and treatment options (including lifestyle changes, pharmacologic therapy, and fundoplication).

Trainees should know the causes and presentation of gastroparesis and be familiar with the indications and limitations of the tests available for the diagnosis of gastroparesis. More specifically, they should know how to interpret nuclear medicine gastric emptying studies and how to integrate information about gastric emptying in the evaluation and treatment of the child with nausea, vomiting, and other dyspeptic symptoms. They should know the treatments available for gastroparesis including lifestyle changes, medications (indications and adverse effects), and the role of surgery.

Trainees also should be familiar with the causes of pseudoobstruction, its presentation, and the available tests to diagnose pseudoobstruction. In addition, they should understand the prevalence and role of small bowel bacterial overgrowth in motility disorders.

Trainees in pediatric gastroenterology should be able to recognize normal and abnormal defecation patterns in children, from newborns to adolescents. Trainees should understand the causes of chronic constipation and fecal incontinence and know when diagnostic studies are indicated. They should understand the approach to treatment, including lifestyle changes, diet, and medications.

Trainees should be familiar with Hirschsprung disease, including the following:

* Pathophysiology

* Epidemiology

* Diagnostic approach – barium enema, anorectal manometry, and suction and full-thickness rectal biopsy (including how to recognize ganglion cells on histological sections)

* Surgical approach and complications

Trainees should understand manometry procedures and their application to disorders of transit, as follows:

* Indications

* Technique of performing

* Risks and shortcomings

* How to recognize tracings consistent with achalasia and Hirschsprung disease (including identification of the recto-anal inhibitory reflex)

Trainees should be aware of the effect of systemic endocrinopathies on GI motility and possible contribution to disorders of transit.

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Patient Care Experiences/Skills

Trainees should be proficient in performing complete histories and physical examinations of patients with FGID or suspected motility problems. They should be able to identify and evaluate GI symptoms, nutritional issues, psychosocial concerns, and other relevant findings.

Trainees should participate in the care of a sufficient number of children with swallowing problems, feeding difficulties, FGID, GERD, constipation, and motility disturbances to be exposed to a wide array of presentations, complications, and therapeutic interventions. They should have the opportunity to participate in the evaluation of children with Hirschsprung disease and collaborate with colleagues in radiology and surgery in diagnosis and management. They should also participate in the care of children with pseudoobstruction.

Trainees should be able to apply and synthesize appropriate evaluation plans, including the following:

* Laboratory studies

* Radiologic imaging

* Endoscopy and colonoscopy

* Motility studies

* Mucosal biopsies

Trainees should understand the complexity of caring for children with FGID and motility disorders and recognize the importance of a multidisciplinary approach to the problem. Trainees should participate in patient care with teams including but not limited to social workers, psychologists, nutritionists, neurologists, surgeons, pathologists, nurses, and motility specialists. Trainees also should play an active role in the nutritional management of children with pseudoobstruction or motility disorders that require enteral nutrition via tubes or parenteral nutrition. Trainees should participate in the long-term care of children with FGID and motility disorders, including continued consultation with other medical specialists as required.

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Examples of Relevant Competencies—Functional GI and Motility Disorders
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GI Bleeding

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Task Force Members

Marsha Kay, Chair

Bradley Barth

Mark A. Gilger

Michael Nowicki

David A. Piccoli

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Importance of Area

GI bleeding occurs not uncommonly in children, is often worrisome, and is occasionally life threatening. Understanding the different etiologies by patient age, the importance of assessing the severity of bleeding, and methods for accurately determining the cause and site of bleeding are critical in the proper management of this condition. Individuals caring for patients with GI bleeding must be able to identify and respond to signs of hypovolemic shock and impending decompensation to prevent ongoing blood loss and avoid a medical emergency.

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Medical Knowledge

Trainees in pediatric gastroenterology should comprehend the differential diagnosis of, diagnostic techniques for, and treatment of GI bleeding, as well as have a thorough understanding of the pathophysiology. The potential sources of UGI and LGI bleeding are listed by age group in Tables 1 and 2.

TABLE 1. Differential diagnosis of acute UGI bleeding in pediatric patients

TABLE 2. Differential diagnosis of acute LGI bleeding in pediatric patients

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With regard to acute GI bleeding, trainees in pediatric gastroenterology should understand the following:

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* The initial and subsequent assessment of a patient with acute GI bleeding, including the signs of impeding decompensation or shock

* The requirements for fluid resuscitation and medical treatment of hemorrhagic shock

* The need to focus on the location and severity of the bleeding

* The components of the preendoscopic evaluation and understand the need for the patient to be hemodynamically stable before endoscopic evaluation

* The elements of postprocedure care, including prevention and assessment of ongoing blood loss

Trainees in pediatric gastroenterology should be familiar with the differential diagnosis and diagnostic approach to the pediatric patient with subacute, intermittent, or chronic GI bleeding from either an upper or lower tract source, including the appropriate use of upper endoscopy and/or colonoscopy as both a diagnostic and therapeutic tool. In the evaluation of bleeding of obscure origin, trainees should comprehend the appropriate use of techniques such as capsule endoscopy, small bowel enteroscopy, and radiologic imaging (eg, nuclear medicine, angiography).

Trainees should understand the appropriate endoscopic assessment and treatment of GI bleeding and that esophagogastroduodenoscopy (EGD) is usually indicated for assessment of acute UGI bleeding requiring transfusion or unexplained recurrent bleeding and can determine the source of the bleeding in most cases from an upper tract source. Trainees must recognize that because UGI bleeding in children frequently stops spontaneously, emergency endoscopy is indicated only when the findings will influence a clinical decision, such as the need for medical or surgical therapy, or if endoscopic therapy can be performed that will stop the ongoing bleeding or prevent rebleeding.

Trainees should comprehend the distinction between lesions amenable to endoscopic therapy (eg, polyps, ulceration, angiomata) and bleeding lesions likely to require surgical therapy to terminate the bleeding episode (eg, Meckel diverticulum, malignancies). Trainees should understand the risks, benefits, applications, and limitations of these techniques, including electrocoagulation (eg, heater probe, monopolar probe, multipolar electrocoagulation probe), argon plasma coagulation, injection of hemostatic agents, band ligation, and mechanical clipping. Trainees should understand that because there is little published experience with these techniques in children, the best technique for each type of bleeding has not been established. Trainees should be aware that the combination of injection (using hemostatic agents such as epinephrine) and thermocoagulation or other mechanical techniques appears to be the most effective endoscopic treatment of UGI bleeding resulting from gastric or duodenal ulcers in children.

Trainees should understand that endoscopic treatment of esophageal varices includes injection sclerotherapy, variceal banding, or a combination of these techniques, and they should know the efficacy and complications of each technique. This should include knowledge that complications of injection sclerotherapy comprise strictures, recurrence of the varices, and recurrent bleeding and that band ligation is becoming the preferred method in both pediatric patients and in adults, is better tolerated in children than sclerotherapy, and has fewer complications (eg, retrosternal pain, fever). Trainees must understand that banding equipment has not yet been adapted for use in infants and small children.

In the setting of acute GI bleeding, trainees must understand the potential benefits and risks of medical therapy, including the following:

* Administration of selective vasoconstrictors (eg, somatostatin analogues), including dosing and duration of therapy

* The role of H2-receptor antagonists and proton pump inhibitors

* Appropriate use of antibiotics in patients with acute bleeding, especially those with cirrhosis, underlying cardiac disease, and other conditions that are associated with an increased risk of infections during a bleeding episode

Trainees should comprehend the value of the various surgical treatment options, including the following:

* Exploratory laparotomy, which is generally reserved for uncontrollable bleeding

* Procedures for bleeding originating from a posterior duodenal ulcer with arterial bleeding, bowel perforation with bleeding, gastroesophageal variceal bleeding, or GI bleeding originating from malignancy

* Portosystemic shunting procedures (mesocaval, distal splenorenal, or central portocaval shunt)

* Esophageal transection, in which the distal esophagus is transected and then stapled back together after varices have been ligated, or devascularization of the gastroesophageal junction (Sugiura procedure), a rare but potentially lifesaving surgery for bleeding esophageal varices

Significant advances in the field of interventional radiology for GI bleeding for both pediatric patients and adults allow for both rapid diagnosis and therapy of many types of bleeding lesions. Trainees should be familiar with the risks, benefits, applications, and limitations of angiographic and other imaging/interventional methods for the diagnosis and therapy of GI bleeding in pediatric patients, particularly when bleeding is obscure in origin or refractory to endoscopic therapy. Trainees should understand the indications, contraindications, diagnostic yield, and relative merits of the following procedures:

* Meckel's scan

* Nuclear medicine bleeding scan (tagged red blood cell scan)

* CT angiography

* Diagnostic and therapeutic angiography (including embolization and coiling, and the radiologic transjugular intrahepatic portosystemic shunt insertion)

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Patient Care Experiences/Skills

Trainees should understand how to obtain a history directed at assessing the rate, severity, and location of the bleeding, and how to identify possible etiologies of GI bleeding. They also should be able to perform a physical examination to evaluate the hemodynamic status of the patient and identify mucocutaneous, abdominal, or other physical examination findings that may suggest the etiology of the bleeding.

Trainees should be competent at performing upper endoscopy and colonoscopy and selected therapeutic procedures. They should be able to identify endoscopic lesions that may result in blood loss. Although the number of therapeutic procedures may be insufficient to obtain competency, opportunities for learning these procedures can take place at hands-on courses. Detailed competencies in terms of procedure skills including methods of assessment are outlined separately in this document (See “Training in Endoscopy and Related Procedures”).

Trainees should participate in the management of patients of different ages with GI bleeding and be able to integrate endoscopic findings, including results of capsule endoscopy and small bowel enteroscopy, as well as those from radiologic procedures, into a management plan for diagnosis and therapy.

Trainees should understand the team approach to the diagnosis and management of GI bleeding and participate in clinical decision making with radiologists, surgeons, and intensivists.

Trainees should have the opportunity to do a follow-up evaluation of patients with a history of GI bleeding and provide counseling regarding possible recurrent bleeding.

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Examples of Relevant Competencies—GI Bleeding
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GI Infections

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Task Force Members

Nicola L. Jones, Chair

Alessio Fasano

David R. Mack

Phillip I. Tarr

Eytan Wine

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Importance of Area

Infections of the GI tract are an important subset of GI disorders to which the pediatric patient is particularly predisposed. Infections also can mimic other GI disorders, such as IBD, and vice versa. Trainees in pediatric gastroenterology, therefore, require comprehensive exposure to the diagnosis and treatment of bacterial, viral, and parasitic infections and their complications.

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Medical Knowledge

Trainees in pediatric gastroenterology should understand the basic pathophysiology, including host defense mechanisms against enteric infections and factors that determine microbial virulence. Trainees should comprehend how congenital or acquired immunodeficiencies can alter the host response to GI infections.

Trainees in pediatric gastroenterology should have an understanding of the epidemiology, natural history, and complications associated with Helicobacter pylori infection and a thorough knowledge of the indications and current recommendations for the testing and treatment of H pylori infection.

Trainees should understand the clinical manifestations, including extraintestinal manifestations, and the complications, diagnosis, and treatment of bacterial infections that cause diarrhea, including the following:

* Campylobacter

* Salmonella

* Shigella

* Yersinia

* Escherichia coli (enterotoxigenic, enteroadherent, enteroinvasive, enteroaggregative, adherent invasive, and Shiga toxin producing)

* Clostridium difficile (including an approach to antibiotic-associated diarrhea)

* Vibrio

* Listeria

Trainees should be familiar with the signs and symptoms of small bowel bacterial overgrowth, as well as its predisposing factors, the available diagnostic tests, and currently recommended therapies.

The pediatric gastroenterology trainee should be aware of potential emerging pathogens and should be able to recognize illnesses related to toxin exposure (eg, staphylococcal food poisoning). They should comprehend the pathophysiology of bacterial-mediated diarrhea and know how to develop a management plan, including the rationale for the use or avoidance of antibiotics, physiologic basis and use of oral/intravenous rehydration solutions, and the advantages and disadvantages of adjunctive therapy (eg, probiotics). Trainees also should be aware of the public health issues related to infections from these organisms. Trainees also should be able to identify and diagnose infections that can resemble Crohn disease (CD).

Trainees should be familiar with the epidemiology, clinical manifestations, prevention, and therapy of viral enteric pathogens (eg, rotavirus; caliciviruses, including noroviruses, adenoviruses, and astroviruses). Trainees also should be familiar with cytomegalovirus colitis and the manifestations, diagnosis, and treatment of Epstein-Barr virus–related complications, particularly in the immunocompromised patient.

Trainees should be familiar with the clinical manifestations, diagnosis, and treatment options for common parasitic infections, including giardiasis, Entamoeba histolytica, and cryptosporidium. They should comprehend the controversies surrounding the pathogenicity of other agents (eg, Dientamoeba fragilis, Blastocystis hominis).

Trainees should understand the primary and secondary immunodeficiencies with GI manifestations and the pathogens, including fungal agents, such as Candida albicans, encountered under these circumstances. Trainees should be familiar with the sexually transmitted diseases that can affect the GI tract.

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Patient Care Experiences/Skills

Trainees should develop expertise in performing a history and physical examination aimed at identifying potential enteric infections or immunodeficiencies with GI manifestations. This expertise can be achieved by working with patients with GI infections in both inpatient and outpatient settings. Where specific enteric infections or immunodeficiencies are rare and unlikely to be encountered, case-based teaching, multidisciplinary case conferences, simulations, and learner-directed reading can be used to ensure that adequate medical knowledge is achieved.

Trainees should become experienced in performing diagnostic testing in patients and should understand the indications and limitations of testing for enteric infections, including being able to use procedures to exclude infections. Trainees should be able to interpret laboratory, radiological, endoscopic, and histological findings. Pediatric gastroenterology trainees should participate in pathology and radiology joint management conferences when possible to enhance knowledge development.

Because of the varied clinical manifestations and need for specialized testing, the management of patients with GI infections often requires multidisciplinary interactions with pathologists, microbiologists, infectious disease specialists, and immunologists. Trainees should be involved in coordinating these multidisciplinary consultations for patients when indicated (eg, suspected immunodeficiency presenting with enteric infection).

Trainees also should participate in the treatment of children with acute and chronic enteric infections to develop the necessary skills to initiate and implement the appropriate treatment.

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Examples of Relevant Competencies—GI Infections
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Hepatology

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Task Force Members

Michael R. Narkewicz, Chair

Regino P. Gonzalez-Peralta

M. James Lopez

Elizabeth Rand

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Importance of Area

Because diseases of the liver and biliary tract have a significant impact on children, trainees in pediatric gastroenterology should have comprehensive exposure to the diagnosis and treatment of these disorders and their complications, as well as a thorough understanding of their pathophysiology.

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Medical Knowledge

Trainees in pediatric gastroenterology should understand the normal structure and function of the liver and biliary tree, and the prevalence, natural history, age-appropriate differential diagnoses, and genetic and other risk factors of hepatobiliary disorders. Caring for children with hepatobiliary disease requires a thorough understanding of both common and rare causes, including infectious, metabolic, genetic, anatomic, immunologic, and toxic (Table 1).

TABLE 1. Categories of hepatobiliary disorders

Trainees should understand the varying clinical manifestations of these disorders, especially the issues that are unique to pediatric hepatobiliary disorders (eg, effects on growth, nutrition, puberty, psychosocial functioning).

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Trainees also should have a complete understanding of the diagnostic criteria that distinguish liver and biliary diseases with common patterns of presentation (eg, cholestasis, neonatal cholestasis, elevated aminotransferases, hepatomegaly, hepatosplenomegaly, acute liver failure, direct or indirect hyperbilirubinemia, ascites), including differences in the following:

* Affected populations

* Results of laboratory evaluations

* Histopathology

* Radiologic studies

* Disease course

Trainees should understand the comprehensive evaluation of patients suspected to have hepatobiliary diseases, as well as the appropriately targeted evaluation of patients with known liver disease who may be experiencing an exacerbation or complication or who require routine monitoring (eg, recommendations for laboratory and radiologic surveillance for cancer). They should understand both the indications for and potential complications of the various tests that may be required. Knowledge of the indications for performance and interpretation of liver biopsy, percutaneous transhepatic cholangiography and ERCP is a key component of the evaluation of hepatobiliary disease. Trainees should know an approach to liver histopathology and be able to recognize the findings in the common pediatric liver diseases, such as biliary atresia, Alagille syndrome, idiopathic neonatal cholestasis, autoimmune hepatitis, sclerosing cholangitis, chronic viral hepatitis B and C, nonalcoholic fatty liver disease, Wilson disease, metabolic liver diseases (eg, glycogen storage disease, mitochondrial disorders, disorders of fatty acid metabolism), α-1-antitrypsin deficiency, cystic fibrosis, hemochromatosis, and hemosiderosis. Imaging studies (eg, abdominal ultrasound [US], CT scan, MRI, MRCP, and radionuclide cholescintigraphy) also are key components of this evaluation. They also should understand the indications and complications for radiologic intervention, such as hepatic abscess drainage, percutaneous transhepatic cholangiography, and transjugular intrahepatic portosystemic shunt.

Trainees should understand the inheritance patterns of the common genetic disorders of the liver (eg, α-1-antitrypsin deficiency, glycogen storage disease type 1A, Wilson disease, Alagille syndrome), use of genetic testing for diagnoses (eg, in Alagille syndrome, hemochromatosis, Gilbert syndrome, α-1-antitrypsin deficiency, Wilson disease, progressive familial intrahepatic cholestasis disorders), and recommendations for screening of family members (eg, in Wilson disease, in hemochromatosis).

Trainees should understand the management of various acute and chronic liver and biliary diseases and their complications. They should fully comprehend the indications for specific medical interventions in the following diseases/disorders:

* Chronic viral hepatitis

* Autoimmune hepatitis

* Biliary atresia and other biliary anomalies

* Cholestasis from other etiologies

* Alagille syndrome

* α-1-antitrypsin deficiency

* Wilson disease

* Nonalcoholic fatty liver disease

* Primary sclerosing cholangitis

* Gallstone disease and other disorders of the gallbladder

* Other metabolic liver diseases

Trainees should understand the indications for specific treatments of in both newly diagnosed and existing hepatobiliary diseases, including the use of the following:

* Enteral and parenteral nutritional therapy

* Fat-soluble vitamins

* Antibiotics

* Chelation therapies

* Corticosteroids

* Immunomodulators

* Antiviral agents

* Antipruritics

Trainees must understand the efficacy, dosing, adverse effects (eg, growth effects of steroids, renal toxicity of calcineurin inhibitors, common interactions of calcineurin inhibitors with other medications, increased infection risk from Epstein-Barr virus and cytomegalovirus with immunomodulators, hypertension with steroids and calcineurin inhibitors, bone marrow suppression with azathioprine, and posttransplant lymphoproliferative disease), and necessary monitoring when using these medications, particularly regarding the use of immunosuppressant and chelation therapies.

Trainees should be familiar with the complications of acute liver failure and their management, including coagulopathy, encephalopathy, renal and metabolic impairment, and increased risk of infection.

Trainees should know the complications of chronic liver disease and their management, including fat-soluble vitamin deficiency and pruritus in cholestasis, coagulopathy, sequelae of portal hypertension (ascites, variceal hemorrhage, and encephalopathy), hepatorenal syndrome, hepatopulmonary syndrome, nutritional complications, and risk and indications of screening for hepatocellular carcinoma.

Although not all trainees will be directly involved in the care of children who are awaiting or have undergone liver transplantation, they should know the indications, approach to the evaluation, outcomes, and acute complications of liver transplantation, especially in the following disorders: biliary atresia, acute liver failure, acute severe Wilson disease, and other metabolic liver disease such as defects of urea cycle metabolism. They also should understand the long-term complications and management issues that arise in these children.

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Required Patient Care Experiences/Skills

Trainees should be able to take a thorough history and perform a physical examination of patients with hepatobiliary disease or suspected disease. Trainees should know how to identify symptoms of liver or biliary tract disease, complications of disease, nutritional concerns, psychosocial concerns, and other relevant findings. Trainees should participate in the care of a sufficient number of patients to be exposed to a wide array of presentations, complications, and therapeutic interventions.

Trainees should be able to establish and implement an appropriate evaluation plan, which may include laboratory and imaging studies, liver biopsy, and endoscopic procedures. Trainees should understand the indications, risks, and procedures for performing percutaneous liver biopsy. Trainees should be competent to perform endoscopic procedures and identify pertinent findings. Trainees should be able to assemble the available data, establish a problem list, and assign a diagnosis/diagnoses or define the next steps in the evaluation.

Trainees should then develop a management plan, with consideration of both specific therapy if available (immunosuppression for autoimmune hepatitis) or general management (optimize nutrition, management of complications such as ascites, and encephalopathy). This treatment plan should include not only the primary diagnosis (eg, biliary atresia) but also any related or unrelated problems (eg, anemia, malnutrition, ascites, pruritus, psychosocial concerns). The plan also should consider the natural history of the primary disorder.

Not all pediatric gastroenterologists participate directly in immediate postoperative management in patients who have undergone liver transplantation. Additional training may be needed for trainees whose career plans require extensive expertise in the care and management of children needing a liver transplant.

In addition to the oversight provided by pediatric gastroenterologists, trainees should experience working with surgeons, radiologists, pathologists, dietitians, psychologists, and others who are expert in these fields. When liver biopsy is necessary, any results should be reviewed with a pathologist with experience in hepatobiliary diseases. Trainees also should review any radiology studies and compare their findings with those of the attending radiologist.

Trainees should understand the multidisciplinary care of patients with hepatobiliary disorders and the role of each team member in that process. This should include interaction with surgeons, radiologists, pathologists, dietitians, and psychologists/social workers, as well as collaboration with other specialists. Trainees should understand the role of surgical management and liver transplantation, including appropriate timing of interventions and options for these patients, and should collaborate closely with their surgical colleagues in this process.

Trainees should continue to be involved in the care of hepatobiliary patients, both outpatient and hospitalized patients, during the course of their 3-year training program. The main goal of this continuity is to learn about natural history of the disease, the management of disease exacerbations and complications, and to monitor for complications. They also should be encouraged to develop collaborative arrangements with other specialists who can help provide optimal care for these patients. Given the chronic nature of many of the hepatobiliary disorders, patients with these diseases will require continued expert medical care into adulthood. As such, trainees should be able to follow the sequence of steps to transition their patients’ care from the pediatric to the adult gastroenterologist.

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Examples of Relevant Competencies—Hepatology
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IBD and Immunologic Diseases

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Task Force Members

Marian D. Pfefferkorn, Chair

Wallace V. Crandall

Michael Kappelman

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Importance of Area

The IBDs, represented by CD, ulcerative colitis, and IBD-unspecified, are common, complex disorders treated frequently in pediatric gastroenterology practices. Advances in mucosal immunology and mechanisms of inflammation continue to shed new light on immune-mediated diseases such as autoimmune gastroenteropathy, celiac disease, allergic/eosinophilic gastroenteropathy, eosinophilic esophagitis, and GI manifestations of immune deficiency. Trainees in pediatric gastroenterology, therefore, require comprehensive exposure to the diagnosis and treatment of these disorders and their complications and a thorough understanding of their pathophysiology.

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Medical Knowledge

Trainees in pediatric gastroenterology should understand the epidemiology, natural history, and complications of IBD, including the pediatric prevalence of these disorders, and the evolving literature on genetic and serologic risk factors. They also should be familiar with the current evidence regarding the pathophysiology of IBD, including the role of genetics, mucosal immunology (both innate and acquired), and the fecal microbiome.

Trainees should understand the varying clinical manifestations of these disorders. Trainees should be able to recognize typical and atypical GI symptoms of IBD, including extraintestinal presentations (growth failure, perianal disease, arthritis, osteopenia/osteoporosis, and sclerosing cholangitis). Trainees should comprehend the issues that are unique to pediatric IBD, namely, its effects on growth, nutrition, puberty, and psychosocial functioning.

They also should have a complete understanding of the diagnostic criteria that distinguish the various types of IBD, including differences in disease distribution, histology, and disease behavior. Trainees should be able to distinguish IBD from other disorders that can have similar symptoms such as irritable bowel syndrome and other functional GI disorders, infection, and autoimmune disorders.

Trainees must be familiar with the comprehensive evaluation of patients suspected to have IBD and an appropriately targeted evaluation of patients with known IBD who may be experiencing an exacerbation or complication, or who require routine monitoring (eg, colonoscopic surveillance for cancer). They should understand both the indications for and potential complications involved with the various tests that may be required. They should understand the components of an evaluation, including the following:

* Laboratory studies (eg, complete blood count, sedimentation rate, C-reactive protein and albumin, and stool studies for pathogens, white blood cells, and calprotectin or lactoferrin)

* Endoscopic evaluation, including EGD and colonoscopy

* Bowel imaging

* Small bowel series

* CT scan

* MRI

* Tagged white blood cell scans

* Capsule endoscopy

Trainees should thoroughly understand the treatment of IBD and its complications, including indications for specific medical interventions in pediatric IBD, and differences in treatment based on disease type (eg, CD vs ulcerative colitis), disease severity (eg, mild vs moderate to severe), and disease behavior (eg, IBD vs penetrating CD)

Trainees should understand the treatment of both newly diagnosed and existing patients with IBD (including the use of nutritional therapy, mesalamine, antibiotics, corticosteroids, immunomodulators, and biologic agents). Trainees must be familiar with the efficacy, dosing, adverse effects, and necessary monitoring when using these medications, particularly regarding the use of immunosuppressant medications. They should recognize the indications for surgical intervention and understand the types of surgical procedures and possible surgical complications, including pouchitis and its management. Trainees must be familiar with guidelines for cancer surveillance in the setting of chronic intestinal inflammation.

Trainees should comprehend the primary and secondary immunodeficiencies with GI manifestations, including familiarity with the innate and acquired immune mechanisms and other mucosal protective factors that operate in the normal host, as well as the clinical manifestations and diagnostic evaluation for disorders caused by deficiencies in mucosal immunity. Specific clinical entities of importance include the following:

* Antibody deficiencies

* Severe combined immunodeficiency and other defects affecting B and T cells

* Chronic granulomatous disease and other disorders of phagocytosis

* Immunodeficiencies associated with other defects

Trainees also should be aware of the secondary causes of immunodeficiency, including human immunodeficiency virus.

Trainees should have a basic knowledge of the epidemiology, clinical features, and diagnostic evaluation of the following systemic autoimmune and vasculitic processes:

* Hemolytic uremic syndrome

* Henoch-Schonlein purpura

* Behçet disease

* Kawasaki disease

* Dermatomyositis

* Mixed connective tissue disease

* Autoimmune enteropathy (either in isolation or as part of a systemic autoimmune disorder such as the autoimmune polyendocrine syndromes)

Trainees should understand the epidemiology of celiac disease and the numerous intestinal and extraintestinal symptoms and signs associated with this condition. Trainees should have expertise in the interpretation of serological testing and human leukocyte antigen typing. Knowledge regarding endoscopic evaluation, dietary management (eg, gluten-free diet), long-term follow-up, and screening for and managing disease-associated comorbidities and complications also are required.

Trainees should be familiar with the pathogenic mechanisms of food allergy and the varied GI and extraintestinal clinical features of allergic conditions. Expert understanding of the role of various diagnostic strategies should be developed, including the following:

* Elimination and challenge

* Skin tests

* Radioallergosorbent testing

* Other immunologic tests

* Other gastroenterological tests (eg, endoscopy, colonoscopy, interpretation of histological findings)

Trainees should be familiar with the current classification and guidelines regarding the diagnosis and management of eosinophilic GI disorders. They should be aware of the latest developments in the diagnosis and management of eosinophilic esophagitis.

Trainees should be aware of the presenting signs and symptoms of autoimmune enteropathy and the diagnostic evaluation of this condition, including serum autoantibody testing and the characteristic endoscopic and histological findings. Knowledge regarding the management and prognosis of this condition is required.

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Required Patient Care Experiences/Skills

Trainees should be able to take a complete history and perform a physical examination of patients with IBD or suspected IBD. They should know how to identify GI symptoms and extraintestinal manifestations, and they should be alert to nutritional concerns, psychosocial issues, and other relevant findings in these patients. Trainees should participate in the care of a sufficient number of patients with IBD to be exposed to a wide array of presentations, complications, and therapeutic interventions.

Trainees should be able to perform a history and physical examination aimed at identifying red flags for systemic autoimmune disorders or immunodeficiencies that have GI manifestations, including a careful history of prior infections, a family history of relevant hereditary conditions, and a detailed review of symptoms. Trainees should develop the clinical judgment and proper index of suspicion necessary to initiate a more thorough evaluation of these systemic illnesses when clinically appropriate (eg, younger age, suspicious findings in history).

Trainees should be able to identify patients suspected of having celiac disease and allergic GI disorders, and develop the skills to diagnose, treat, and monitor affected children appropriately. Trainees should recognize the need to coordinate multidisciplinary care when required, including consultation with allergy/immunology specialists and registered dietitians with expertise in the dietary management of allergic diseases.

Trainees should be able to recognize children with intractable diarrhea of infancy and conduct a systematic evaluation for underlying etiologies. Management of specific conditions should be initiated, including both nutritional support and medical therapy.

Trainees should be able to establish and implement an appropriate evaluation plan that may include laboratory studies, stool studies, endoscopic procedures, and radiologic imaging. They should be competent to perform endoscopic procedures in these patients, identify pertinent findings, and consult with a pathologist to review biopsy specimens. Trainees should be able to assemble the available data, assign a diagnosis, and classify the disease type, severity, and behavior.

Trainees should then establish a treatment plan, including both induction therapy and maintenance therapy, that considers not only the primary diagnosis (eg, CD) but also other related or unrelated problems (eg, anemia, malnutrition, fistula, psychosocial concerns, osteopenia). Trainees should continue to care for patients with IBD, both outpatient and hospitalized, during the course of their 3-year training program to learn about evolving phenotypes, the management of disease exacerbations, and monitoring for complications. Trainees should develop collaborative arrangements with other specialists who can help provide optimal care for these patients.

Trainees should understand the indication for endoscopy and colonoscopy in the diagnosis of GI and systemic autoimmune conditions, immunodeficiencies, and allergic disorders, and should develop the clinicopathological perspective necessary to interpret laboratory, radiologic, endoscopic, and histologic findings.

Trainees should understand the multidisciplinary care of IBD, and the role of each team member in that care, which should include interaction with dietitians, radiologists, pathologists, and psychologists/social workers. Care also may require collaboration with endocrinologists, specialists in bone metabolism, and others. Trainees should understand the indications for surgical management, appropriate timing of surgical interventions, and the surgical options for these patients, including ostomies and their care, and should collaborate closely with their surgical colleagues.

Given the chronic nature of IBD and certain other immune-mediated diseases, trainees should have the opportunity to provide longitudinal care. Furthermore, trainees should become familiar with the sequence of steps involved in the transition of care from the pediatric to the adult gastroenterologist.

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Examples of Relevant Competencies—IBD and Immunologic Diseases
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Malignancies and Premalignant Conditions of the GI Tract

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Task Force Members

Steven H. Erdman, Chair

Carol Durno

Victor L. Fox

Sherry C. Huang

Warren Hyer

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Importance of Area

Although the hereditary GI cancers and polyposis syndromes are relatively rare, they can be associated with significant morbidity for the patient and family. Because of this, the pediatric gastroenterologist plays a critical role in the diagnosis and management of patients with these disorders. An accurate diagnosis is vital to determining the prognosis and future surveillance of these children and therefore has serious medical implications for the family.

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Medical Knowledge

Trainees in pediatric gastroenterology must understand the molecular genetics of hereditary GI cancer, including inheritance patterns, disease phenotypic expression, and natural history. They also must know that for autosomal dominant conditions, other family members, including parents and siblings, are at risk and should participate in genetic evaluation and screening. Understanding the importance of a multidisciplinary approach to the evaluation, diagnosis, and management of malignant and premalignant conditions of the GI tract that includes the expertise of a cancer genetics counselor, a geneticist, social worker and surgeon, is critical.

Trainees in pediatric gastroenterology should be familiar with the types and locations of malignancies of the GI tract and liver and their relative importance throughout infancy and childhood. They also should be aware of the specific presenting signs and symptoms of GI malignancies, including hormone-secreting tumors of childhood that can be of GI origin, such as carcinoid tumors, insulinomas, pheochromocytomas and other neuroendocrine tumors. With regard to each type of malignancy, they should understand how to formulate an age-specific differential diagnosis for these lesions, the medical and surgical management options, and the strategies used to detect recurrence and prevent complications.

Trainees also should have knowledge of the diseases and medical circumstances of childhood that predispose to malignancy of the GI tract, liver, and pancreas, including the following:

* Adenomatous polyp syndromes

* Hamartomatous polyp syndromes

* Other syndromes associated with the development of colon cancer

* Disorders/syndromes associated with liver tumors

With regard to polyps and polyposis syndromes, trainees should have an understanding of the following:

* The histologic criteria that differentiate adenomas from hamartomas

* The significance of GI adenomas as premalignant lesions

* How to differentiate patients with simple hamartomas from those who require additional evaluation based on family history, polyp number, or location

* The phenotype of each of the polyposis syndromes, including the genetics, natural history, extraintestinal findings, and psychosocial consequences of these diseases

* The role of endoscopic polypectomy

* The indications and timing of prophylactic colectomy when used to manage the polyposis syndromes

* The surgical options and any potential complications of these options

Trainees should recognize the importance of lifelong surveillance in the identification of new/recurrent disease or disease complications and the impact of noncompliance on these patients.

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List of Required Patient Care Experiences/Skills

Trainees should be able to take a careful history, including a detailed family history, and be able to recognize the red flag features that warrant further evaluation. They also should be familiar with the indications, expected outcomes, and limitations of genetic testing and understand the importance of informed consent/assent as part of a genetic evaluation.

Trainees should be able to identify the important physical findings associated with the polyposis syndromes, including skin lesions, cutaneous and abdominal masses, and the like.

Trainees should be comfortable with the use of the following state-of-the-art imaging modalities of the abdomen and intestinal tract for diagnosis:

* UGI series

* CT and MRI

* Nuclear medicine studies

* Capsule endoscopy

Trainees should be competent in upper endoscopy and colonoscopy, both for diagnosis and therapy, including the following:

* Differentiating sessile from pedunculated polyp lesions

* Endoscopic biopsy, polypectomy, and techniques for polyp/biopsy retrieval (eg, brushings, aspirates)

* Managing the complications associated with polyp removal

Trainees must have the opportunity to review biopsies with a pathologist who has expertise in GI malignancy.

Trainees must understand how to evaluate and manage the GI complications of patients receiving therapy for cancer, including bone marrow transplantation.

Because these disorders are uncommon, trainees should use every opportunity to participate in the long-term care of children with polyposis syndromes.

Trainees should master the skills of collaborative patient management and be able to use the expertise and abilities of other team members, including genetic counselors, geneticists, surgeons, pathologists, and social workers, in the diagnosis and multidisciplinary management of malignant and premalignant conditions.

Trainees should recognize the importance of regular follow-up care and surveillance for patients with these disorders. Transition of care from pediatric to adult health care providers is of the utmost importance to ensure continuity and ongoing follow-up treatment for these patients.

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Examples of Relevant Competencies—Malignancies and Premalignant Conditions of the GI Tract
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Nutrition

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Task Force Members

Mark Corkins, Chair

John A. Kerner Jr

Linda Muir

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Importance of Area

Because the primary function of the GI system is as the conduit for nutrient intake, the processes involved in nutrition must be one of the core knowledge sets for the pediatric gastroenterologist.

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Medical Knowledge

Pediatric gastroenterology trainees should understand the physiology involved in digestion, absorption, and metabolism of the entire range of nutrients. In addition, they should appreciate the changes in digestion and metabolism that occur during development from neonate to young adult.

Trainees should have a basic understanding of the factors that drive and affect a person's oral intake and the effect that various diseases have on appetite and intake. Knowledge of the development of the oral cavity, the basics of mastication, including teething and chewing, and the mechanisms of swallowing is essential.

Trainees should comprehend the gastric process of homogenization of ingested food with the gastric secretions and the “grinding action” of gastric contractions to reduce particle size as preparatory steps of digestion.

Pediatric gastroenterology trainees should understand the small intestinal processes of digestion of macronutrients (protein, fat, and carbohydrate), including the following:

* Function of digestive enzymes: salivary, gastric, intestinal mucosal (disaccharidases), pancreatic, and breast milk enzymes, with regard to site of activity, specific substrates, storage, method of activation, necessary cofactors, products of digestion

* Role of bile acids

* Method of absorption and transport of products of digestion and subsequent cellular processing, if required

* Intestinal location where digestion and absorption occur for different nutrients

* Whether the product of digestion is immediately used, stored, or processed to another form

Trainees also should have an understanding of micronutrient (vitamin and mineral) absorption, including the following:

* The specifics of the site of absorption, carriers, transporters, and subsequent cellular processing

* Dietary agents that can enhance or block absorption

* The postabsorption path of the micronutrient, any further modifications, and the subsequent use or storage of the micronutrient

A crucial nutritional concept for pediatric gastroenterology trainees is the dynamic base daily nutritional requirements for the growing and developing child, including the following (in order of importance):

* Daily fluid needs and the difference between total and intravascular fluid volumes (the most basic information)

* Daily caloric needs

* Basic requirements for protein and fat

Trainees need to know the appropriate requirements for calories, protein, and fat based on the age, nutritional status, and diagnosis of the child, and the recommended daily intakes for vitamins and minerals, as well as the changes that occur in the recommended daily intakes with age and the alterations caused by various diseases. Trainees should be aware of the disease states that can increase nutrient losses and result in deficiencies. Trainees also should comprehend the manifestations and complications of nutritional deficiencies and toxicities. The potential effect of medications on nutrient requirements also should be known.

The diseases associated with inadequate and excess nutrition intake should be understood. The signs and long-term consequences of malnutrition, including kwashiorkor, should be recognized. Fellows should know the physiology of and approach to refeeding syndrome. Understanding the consequences of excess nutrition intake and resultant obesity are crucial for pediatric gastroenterology trainees. Trainees should be familiar with the evaluation of overweight and obese children, the complications of obesity, and the spectrum of options for management, including bariatric surgery for selected adolescents.

Trainees should be able to estimate the nutritional needs of the patient using published recommendations and predictive equations, and synthesize that with a working knowledge of resting and total energy expenditure and how this is affected by body composition. Trainees should be aware of how disease can alter nutritional needs in patients and be able to formulate individual nutritional interventional plans, using the appropriate level of intervention for each patient's disease and nutritional status and applying longitudinal assessment, growth measures and anthropometrics, to monitor the patient's ongoing nutritional status.

Trainees should be familiar with the nutritional content of breast milk, infant formulas, and the enteral nutrition products used in pediatrics. Trainees should have a working knowledge of lactation and support of the breast-feeding mother. They also should know the supplements and additives and how to modify breast milk or formulas to meet a recognized nutritional need in a patient. Trainees should understand enteral access devices, their placement (including documentation), care, and complications. Trainees must be able to select the correct formula and know the standard and disease-specific and metabolic-specific formulas. Trainees should know how to administer enteral nutrition, including bolus, and continuous drip, and how to transition feedings. Trainees also should be cognizant of drug–nutrient interactions in patients.

Trainees should understand the indications for parenteral nutrition. They should have a working knowledge of principles of vascular access, including the placement, care, and complications of vascular access devices. Trainees should be familiar with the basic formulations of parenteral nutrition, including the adjustments needed for various disease states. They should be aware of the appropriate monitoring for patients on parenteral nutrition, including acute, chronic, and home administration. Trainees should know the complications of parenteral nutrition, including the following:

* Metabolic complications (including gallstone and bone disease)

* Parenteral nutrition–related liver disease

* Mucosal atrophy

* Micronutrient deficiencies and manifestations

* Central line sepsis and mechanical complications

Pediatric gastroenterology trainees should know the specific nutrition requirements and nutritional interventions in the following disease states:

* Short bowel syndrome

* Cystic fibrosis

* Diarrhea, acute and chronic

* Celiac disease

* Pancreatic insufficiency

* Pancreatitis

* GI allergy

* Hepatobiliary disease

* IBD

* Functional bowel disorders

* Motility disorders

* Immunocompromised disorders

* Eating disorders

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Required Patient Care Experiences/Skills

Pediatric gastroenterology trainees should be able to elicit a comprehensive nutritional history and assess the patient's nutritional status on physical examination.

Trainees should be able to apply other tools to assess nutrition status:

* Growth measures (growth charts, including disease specific)

* Anthropometrics

* Laboratory studies (including interpretation and understanding limitations)

* Indirect calorimetry

* Dual-energy x-ray absorptiometry

Pediatric gastroenterology trainees must have longitudinal experience caring for children with nutritional problems. Trainees need to evaluate and treat patients with feeding problems. Fellows must design nutritional interventions for children with malnutrition and obesity and then monitor the responses over time.

Trainees must prescribe appropriate enteral and parenteral nutrition therapy for patients with nutritional needs. They must request the appropriate monitoring for the therapy they have prescribed.

Trainees should be able to participate as a member of a multidisciplinary team (registered dietitians, pharmacists and nursing) and use the team to provide nutrition support to pediatric patients.

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Examples of Relevant Competencies—Nutrition
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Pancreatic Disease

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Task Force Members

M. James Lopez, Chair

Bradley Barth

Cheryl Gariepy

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Importance of Area

Pancreatic diseases are a combination of congenital, genetic, and acquired disorders that are becoming more prevalent in pediatric GI practice. Therefore, trainees should understand the normal development and physiology of the pancreas and should have a comprehensive knowledge of the diagnosis and treatment of acute and chronic pancreatitis and of pancreatic insufficiency.

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Medical Knowledge

Trainees in pediatric gastroenterology should have a clear understanding of basic pancreatic exocrine physiology and changes with development, including the following:

* Development of pancreatic secretion

* Age-related differences in pancreatic enzyme secretion

* Mechanisms of pancreatic enzyme activation

* Cofactors important for enzyme function

* Hormonal and dietary stimulants of pancreatic secretion

Trainees should comprehend the normal development of pancreatic structures with a particular focus on important clinical variants, such as the following:

* Pancreas divisum

* Annular pancreas

* Pancreatic agenesis

* Pancreatic rests

* Other anatomic variants (eg, congenital cysts, long common channels)

Trainees should be familiar with the clinical presentations that would raise clinical suspicion for these entities and understand diagnosis using imaging and endoscopic techniques and surgical or medical interventions to treat these abnormalities, when needed.

Trainees should be familiar with genetic causes of pancreatic insufficiency, including syndromic disorders (eg, Johanson-Blizzard, Shwachman-Diamond, and Ivemark) and metabolic disorders (eg, Pearson and other mitochondrial disorders, organic acid disorders).

Particular attention should be paid to cystic fibrosis (CF), its epidemiology, natural history, and GI complications, with a strong focus on pancreatic insufficiency. Knowledge of this area should include understanding the evaluation and diagnosis of pancreatic insufficiency, clinical consequences of pancreatic insufficiency, and appropriate treatment strategies, including understanding the appropriate dosing of enzymes and relating this to the normal physiology of enzymatic action.

Trainees should be aware of the specific GI complications of CF and the treatment options for each. This should include knowledge of the natural history of liver disease and portal hypertension and the specific role of liver transplantation and treatments for portal hypertension in CF. They should be aware of fibrosing colonopathy, meconium ileus, distal intestinal obstruction syndrome, rectal prolapse, and acute pancreatitis, including evaluation and treatment options for each of these.

Trainees should understand and be able to develop a comprehensive approach to diagnosis and treatment of acute pancreatitis. This approach requires knowledge of potential causes, including the following:

* Age-appropriate considerations

* Assessment of potential acute complications (eg, hemorrhage, necrosis, shock, pleural effusion, peripancreatic fluid collections, hypocalcemia, lipid abnormalities, insulin insufficiency)

* Clinical (and radiographic) assessment of severity (eg, Atlanta criteria, Ranson's criteria, modified Glasgow scale)

They should be knowledgeable about the potential predictors of prognosis (recognizing that these have not been validated in children) and understand the indications for different methods of nutritional support, treatment with antibiotics, and surgery.

Trainees should understand and develop a comprehensive approach to the diagnosis and treatment of chronic pancreatitis. They should be well versed in the methods of diagnosing morphological changes in the pancreas and for diagnosing pancreatic insufficiency. Particular attention should be paid to the following findings:

* Chronic pain

* Pancreatic exocrine and endocrine insufficiency

* Progressive pancreatic ductal changes and pseudocyst formation,

* The role of MRCP, ERCP, endoscopic ultrasound (EUS), and surgery in the management of these problems

Trainees should be familiar with the influence of genes on pancreatitis (both acute and chronic) and knowledgeable about hereditary or familial pancreatitis, including the following:

* Epidemiology

* Pathophysiology

* Genetic basis

* Natural history and complications

* Potential interventions or treatments for complications

This would include knowledge of appropriate approaches to diagnosis, uses of imaging modalities, and long-term treatment and monitoring. They should understand the role of other genetic risk factors for the development of pancreatitis. Trainees should be taught the ethics and ramifications of genetic testing in patients and their relatives for those diseases.

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Required Patient Care Experiences/Skills

Caring for patients with CF, acute pancreatitis, and chronic pancreatitis by mentored teaching and oversight by pediatric gastroenterologists is desirable. Trainees should be able to perform a complete history and physical examination to identify issues relevant to the clinical problems of acute and chronic pancreatitis, and pancreatic insufficiency (eg, CF). For patients with acute pancreatitis, trainees should be able to provide careful consideration of the following:

* Potential etiologies and complications

* Pain assessment

* Evaluation for potential complications on examination

* Assessment of potential psychosocial or nutritional issues that are relevant (eg, obesity, severity of pancreatitis)

They should be able to establish an appropriate evaluation plan that includes laboratory studies to identify complicating factors, imaging studies, and diagnostic tests. Trainees should be able to define areas of concern or a problem list and develop a clear and specific approach for treatment and evaluation of each entity.

Trainees also should be able to develop appropriate evaluation plans for patients having pancreatic insufficiency and chronic pancreatitis, and they should be able to apply and synthesize the findings for these patients, and provide specific care plans for these patients. Patient care also should include emphasis on appropriate collaborations with surgeons, radiologists, pulmonologists, intensivists, dietitians, mental health professionals, and pain management specialists, as indicated by disease severity or complications.

Trainees should have the opportunity to manage patients with chronic pancreatitis over time and to reevaluate patients with acute or recurrent pancreatitis after the acute episode has subsided.

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Examples of Relevant Competencies—Pancreatic Disease
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Peptic Disease

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Task Force Members

Craig A. Friesen, Chair

Benjamin D. Gold

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Importance of Area

Because acid peptic diseases are among the most common conditions treated in pediatric gastroenterology practice, trainees in pediatric gastroenterology require comprehensive exposure to the diagnosis and management of these conditions and should have a thorough understanding of their pathophysiology.

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Medical Knowledge

Trainees in pediatric gastroenterology should understand the anatomy, physiology, and development of the esophagus, stomach, and duodenum as they relate to acid peptic conditions. They also should have a clear understanding of the pathophysiology of acid peptic disease in the esophagus, stomach, and duodenum, including the following:

* Developmental factors

* Hypersecretory states, such as those resulting from derangements in the secretion of GI hormones

* Disruption of mucosal protective mechanisms

Trainees should understand the natural history, epidemiology, presentation, and complications of acid peptic diseases, including peptic ulcer disease, H pylori–associated gastritis, and GERD. They should be familiar with the extraesophageal manifestations of GERD. Trainees should be knowledgeable regarding differences in presentation of conditions in the differential diagnosis of acid peptic diseases, including the following:

* Functional dyspepsia

* Other causes of vomiting, including serious disorders such as brain tumors

* Eosinophilic esophagitis

* Infectious esophagitis, gastritis

* Other causes of GI tract inflammation (eg, CD, celiac disease)

* Eating disorders

* Symptom falsification (by the patient or the patient's caregiver)

Trainees must be proficient in the evaluation of acid peptic diseases and should have a complete understanding of all diagnostic approaches for acid peptic disorders in children, including indications, contraindications, benefits, costs, limitations, and interpretation. These diagnostic approaches include the following:

* Empiric therapeutic trials

* Esophageal pH and impedance monitoring

* UGI fluoroscopy

* Gastric scintiscans

* Tests for H pylori

* Interpretation of gross endoscopic findings (eg, ulcers, nodular gastritis, eosinophilic esophagitis)

* Mucosal histopathology

Trainees should understand the treatment of acid peptic diseases and their complications and should be knowledgeable about the nonmedical options for treating acid peptic diseases (eg, positioning, lifestyle modifications, pharmacotherapy). Trainees should have a clear understanding of the effects of body weight and age on drug dosing, pharmacology, efficacy, adverse reactions, interactions, and contraindications of medications, including the following:

* Antacids

* H2-receptor antagonists

* Proton pump inhibitors

* Mucosal protective agents

* Prokinetic drugs

* Antibiotic regimens used to treat H pylori

Trainees should comprehend the roles of therapeutic endoscopy and surgery in the treatment of acid peptic disease and their complications. They should be knowledgeable about surgical approaches for the treatment of GERD and endoscopic approaches to the management of complications of GERD, including the various procedures, risks, benefits, and short- and long-term complications.

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Required Patient Care Experiences/Skills

Trainees should be able to take a complete history and perform physical examinations on patients with suspected acid peptic diseases. They should be able to identify clinical symptoms of acid peptic diseases, including typical symptoms of peptic ulcer disease and GERD, as well as symptoms of possible complications, such as respiratory; ear, nose, and throat; and behavioral manifestations of GERD. Trainees should participate in the evaluation and management of a sufficient number of patients with acid peptic disease to be exposed to a wide array of presentations, complications, and therapeutic interventions.

Trainees should be able to establish and implement an appropriate evaluation plan that may include the following:

* Empiric medication trials

* Laboratory studies

* Esophageal pH and/or impedance monitoring

* Endoscopic procedures

* Radiologic imaging

Trainees should perform and interpret esophageal pH and impedance monitoring. They should perform supervised endoscopic procedures in these patients, and identify pertinent findings. In conjunction with the pediatric gastroenterologist, trainees should then assemble the available data, assign a diagnosis, and assess the severity and complications of the disease or disorder. Following assessment and diagnosis, trainees should then establish a treatment plan.

Trainees should be encouraged to collaborate with other specialists who can help provide optimal care for these patients. If a biopsy is indicated, biopsy specimen findings should be reviewed with a pathologist experienced in the interpretation of histopathologic findings in acid peptic diseases. Trainees should review any radiologic studies in conjunction with a radiologist experienced in interpreting these findings in acid peptic diseases. Trainees should participate in combined conferences (eg, GI pathology, GI radiology) to provide additional education regarding these unique aspects of the diagnostic evaluation.

Trainees should continue to be involved in the care of patients with acid peptic disease during their course of their training to learn about the long-term course and manifestations of these conditions, including evolving complications.

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Examples of Relevant Competencies—Peptic Disease
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Short Bowel Syndrome and Intestinal Failure

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Task Force Members

Lesley Jacqueline Smith, Chair

Simon P. Horslen

Samuel Kocoshis

Clarivet Torres

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Importance of Area

Although the congenital and acquired lesions that result in short bowel syndrome (SBS) are rare, children continue to experience this complication, and optimal medical care can make a substantial impact on the child's quality of life. This also is a disorder for which a multidisciplinary approach is critical. Finally, understanding the approach to patients with SBS can be useful in the evaluation and management of other patients with malabsorption or maldigestion.

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Medical Knowledge

Trainees should understand that intestinal failure is the result of an insult to the bowel that occurs either as a result of congenital anomalies (eg, gastroschisis, intestinal atresia, malrotation and volvulus, Hirschsprung disease) or as a result of an acquired catastrophe (eg, necrotizing enterocolitis, mesenteric thrombosis, trauma, CD). The loss of bowel obligates nutrition support for a variable period to allow for adaptation of the intestine. Failure of this process may result in permanent intestinal failure and a long-term requirement for parenteral nutrition and prompt consideration of intestinal transplantation. Trainees should be aware that there are other conditions that result in intestinal failure that require similar multidisciplinary diagnostic and management skills (eg, intestinal pseudoobstruction syndromes, inflammatory disorders), but these are covered elsewhere in these training guidelines.

Trainees should thoroughly comprehend the following:

* Basic embryology and anatomy of the GI tract

* The processes of rotation and fixation of the gut during fetal development

* The physiology, cell biology, immunology, endocrinology, and biochemistry of intestinal absorptive, secretory, motor, and sensory functions and how these change with development

* The regulation of growth of the intestine and of the processes of gut adaptation after acute and chronic injury

Trainees in pediatric gastroenterology should understand the epidemiology and natural history of intestinal failure and recognize the complications of this syndrome, particularly in relation to the overall prognosis for rehabilitation of the gut. Trainees should be aware of the importance of such factors as the following:

* The length of the small intestine

* The physiologic significance of loss of the ileum in terms of adaptation versus loss of the jejunum

* The role of the colon and of the ileocecal valve

* The contribution of bowel obstruction, bloodstream infection, percentage of enteral feeding, and the composition and delivery of parenteral nutrition in the genesis and evolution of cholestasis

Trainees should be familiar with the early postsurgical consequences of the syndrome (eg, fluid–electrolyte imbalance in the ostomy patient), surgical (eg, obstruction, fistulas) and medical complications (eg, hypergastrinemia, total parenteral nutrition [TPN]–associated cholestasis), and the long-term consequences of a short bowel (eg, intestinal failure, nutritional deficiencies, bloodstream infection, bacterial overgrowth, difficult vascular access, GI bleeding, portal hypertension, liver failure). Because patients with intestinal failure require nutritional support, including both parenteral and enteral nutrition using defined formulas, trainees must become thoroughly conversant with the indications for and composition and prescription of parenteral nutrition solutions and formulas as developmentally appropriate.

Trainees should understand the indications, technique, and interpretation of the various diagnostic modalities used in the evaluation and management of the patient with intestinal failure. Diagnostic modalities include the following:

* Endoscopy of the UGI and LGI

* Capsule endoscopy

* Liver biopsy

* UGI and LGI contrast imaging

* CT and MRI scanning

* US techniques, including Doppler imaging

Trainees should comprehend the histopathologic interpretation of surgical specimens, endoscopic mucosal biopsies, and liver biopsies.

Trainees should also be familiar with the medical management of the patient in terms of the provision of nutritional support, prevention of nutritional deficiencies, monitoring and interpretation of growth parameters, and the use of pharmacologic interventions to treat the various complications of SBS. Trainees should have a specific understanding of the management of bacterial overgrowth (including D-lactic acidosis), cholestasis and chronic liver disease, and central venous lines (including prevention and treatment of bloodstream infections).

Because the management of SBS involves close collaboration with the pediatric surgical team, trainees should know the surgical management options for SBS and be thoroughly cognizant of the indications for their use. Relevant surgical procedures include the following:

* Ostomy creation and management

* Bowel reanastomosis

* Fistula management

* Tapering and lengthening operations

* Assessment and management of bowel obstruction

* Vascular access techniques

* Gastrostomy insertion

* Management of strictures (both anastomotic and otherwise)

Trainees also must understand the factors that affect the timing and selection of patients for intestinal transplantation, such as TPN-associated cholestasis, difficult vascular access, and recurrent bloodstream infection. Trainees should be cognizant of the current indications for the various types of intestinal transplantation available and understand the anatomic considerations involved in the choice of operation. Trainees also should be aware of the common complications following transplantation of the bowel, including surgical postoperative issues (eg, bleeding, thrombosis, wound infection, compartment syndrome), rejection, infection, and long-term complications (eg, renal dysfunction, growth failure, hypertension, diabetes, and posttransplant lymphoproliferative syndrome). Trainees should also understand the histopathologic diagnoses of intestinal rejection and opportunistic infections.

Trainees should recognize that the chronic nature of this syndrome requires an understanding of the influence of SBS or intestinal failure on the growth and development of the child, and on the child's functioning within the context of family and society. Care for these patients involves a multidisciplinary hospital and community-based team (eg, GI physicians, general surgeons, transplant surgeons, infectious disease specialists, dietitians, nurse practitioners, pharmacists, social workers, psychologists, home care providers, and rehabilitation specialists [eg, feeding specialists, physiotherapy, occupational therapy]).

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Required Patient Care Experiences/Skills

Trainees should be able to take complete histories and perform thorough physical examinations of patients with intestinal failure in sufficient numbers to allow them to be comfortable and competent in assessing such patients. Trainees should be able to identify all of the relevant problems, including GI symptoms, extraintestinal manifestations (eg, liver disease), and nutritional, growth, and psychosocial concerns. They should have exposure to a sufficient number of patients that they are able to assess underlying pathophysiology and potential complications and decide on appropriate diagnostic and therapeutic modalities.

Trainees should be able to formulate a comprehensive evaluation and treatment plan for the patient that encompasses the complete problem list. Trainees should be able to apply and synthesize the findings from laboratory studies, urine and stool testing, endoscopic procedures, liver biopsy, radiologic imaging, and pathologic interpretation. Supervised by an attending pediatric gastroenterologist or surgeon, trainees should perform endoscopic and biopsy procedures in these patients and identify pertinent findings. Trainees should review all radiology and pathology studies and compare their findings with the official radiology or pathology report.

Trainees should participate in multidisciplinary diagnostic conferences with experts from surgery, radiology, and pathology. They should have the opportunity to lead the multidisciplinary team as their training and level of competence increases. Trainees should then assemble the available data to refine the diagnosis and problem list, classifying it in the order of priority assigned. Trainees should then develop a comprehensive treatment plan, taking into account all of the problems defined on the problem list, ordered as to priority. The treatment plan should therefore encompass both the primary diagnosis (eg, admission for line-associated bloodstream infection in SBS) and other problems (eg, malabsorption, nutritional support, TPN-associated cholestasis, psychosocial concerns).

Trainees should be involved in the care of patients having intestinal failure over time, including both inpatients and outpatients, and they should be allowed to participate in the coordination of the community care of such patients. Treatment and care should be carried out by pediatric gastroenterologists, by all members of the multidisciplinary team, and by families in a comprehensive manner, always based on patient-centered care in the context of the family.

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Examples of Relevant Competencies—Short Bowel Syndrome and Intestinal Failure
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