Pediatric Short Bowel Syndrome Clinical Presentation

  • Author: Carmen Cuffari, MD; Chief Editor: Jatinder Bhatia, MBBS   more...
 
Updated: Mar 22, 2012
 

History

The history of a patient with short bowel syndrome (SBS) is typically of a child who was born with a congenital anomaly, such as an omphalocele, gastroschisis, or intestinal atresia, and who required a small-bowel resection. In addition, premature infants with necrotizing enterocolitis and require extensive bowel resection with or without the loss of the ileocecal junction also contribute to this patient population.[2]

Other patients present with a past medical history of intestinal ischemia from malrotation and volvulus that required intestinal resection. Congenital short bowel syndrome is a rare cause of short bowel syndrome. Over the last several years, the authors have noted numerous patients with short bowel syndrome associated with cloacal exstrophy. Although these children do not have short bowel syndrome in the classical sense, they behave clinically with all signs and symptoms associated with functional intestinal failure.

Children with short bowel syndrome may present with various medical issues, depending on the extent of their bowel resection and the level of medical complexity. The history should consider all the potential clinical ramifications of managing cases of short bowel syndrome, including the following:

  • Parenteral nutrition
    • The degree of home nutrition support necessary in the management of a child on total parenteral nutrition (TPN) is noteworthy.
    • Guidelines for the safe use of parenteral nutrition have been established by the American Society for Parenteral and Enteral Nutrition.[3]
    • Patients may present with issues entirely separate from the medical problems related to short bowel syndrome, including problems associated with intravenous access, infection, and signs and clinical symptoms associated with TPN-related liver disease.
  • Enteral nutrition
    • Quickly initiate enteral feeding for all children with short bowel syndrome.
    • Once again, patients may present with a history entirely separate from the medical problems related to short bowel syndrome, including gastrostomy or nasogastric tube issues. For example, gastrostomy tubes may accidentally fall out. In these patients, the immediate replacement of these tubes is important in maintaining the patency of the tube entry site.
    • Although complications are uncommon, be aware of potential gastric ulceration, gastrostomy tube migrations, and intestinal obstruction, which all may be associated with bilious vomiting and the risk for pancreatitis.
  • Nutrition
    • Closely monitor all children on long-term enteral and parenteral nutrition for specific nutritional issues.
    • Patients may present with various symptoms related to specific nutritional deficiency, including specific vitamin (or mineral) deficiencies and the related signs, symptoms, and electrolyte abnormalities and their potential complications.
  • Medical and surgical histories: Obtain a detailed account of the patient's past medical and surgical histories.
    • The pathology leading up to the surgical resection
    • The extent and location of bowel resection, the presence or absence of the ileocecal valve
    • Medical complications
    • TPN dependency
    • Enteral nutrition
    • Enteral access
    • Type of nutritional formula used
    • Nutritional supplements
    • Medications
    • Allergies
  • History of complications associated with short bowel syndrome
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Physical

During the physical examination, pay close attention to these clinical signs.

  • Vitals
  • State of hydration
  • State of nutrition, as measured by a patient's weight for height and anthropometric measurements
  • Signs of sepsis
  • Form of nutritional therapy used in the patient (eg, central line access or enteral access)
  • Specific clinical signs of nutritional deficiency
  • Signs of liver disease
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Causes

  • Necrotizing enterocolitis, intestinal atresias, and midgut volvulus are the most common causes of short bowel syndrome in the neonatal period.
  • Intussusception with ischemic small-intestinal injury is a common cause of short bowel syndrome in older infants and children.
  • Through innovations in the surgical management of patients with chronic inflammatory bowel disease, Crohn disease is a less frequently associated cause of short bowel syndrome.
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Contributor Information and Disclosures
Author

Carmen Cuffari, MD  Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Jorge H Vargas, MD  Professor of Pediatrics and Clinical Professor of Pediatric Gastroenterology, University of California, Los Angeles, David Geffen School of Medicine; Consulting Physician, Department of Pediatrics, University of California at Los Angeles Health System

Jorge H Vargas, MD is a member of the following medical societies: American Liver Foundation, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David A Piccoli, MD  Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, State University of New York Downstate Medical Center

Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research

Disclosure: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, Inc. Grant/research funds Independent contractor

Chief Editor

Jatinder Bhatia, MBBS  Professor of Pediatrics, Chief, Section of Neonatology, Department of Pediatrics, Medical College of Georgia

Jatinder Bhatia, MBBS is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Dietetic Association, American Pediatric Society, American Society for Clinical Nutrition, American Society for Parenteral and Enteral Nutrition, Society for Pediatric Research, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

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Small-bowel obstruction visible on plain radiograph caused by intussusception in a 5-month-old patient.
 
 
 
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