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Pediatric Short Bowel Syndrome Clinical Presentation

  • Author: Carmen Cuffari, MD; Chief Editor: Jatinder Bhatia, MBBS, FAAP  more...
Updated: Jul 08, 2016


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.[1]

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.[4]
    • 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
    • Malabsorptive diarrhea
    • Dehydration
    • Vomiting
    • Bloating
    • Gastroesophageal reflux
    • Failure to thrive
    • Drug toxicities


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


See the list below:

  • 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.
Contributor Information and Disclosures

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, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching.

Specialty Editor Board

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, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Chief Editor

Jatinder Bhatia, MBBS, FAAP Professor of Pediatrics, Medical College of Georgia, Georgia Regents University; Chief, Division of Neonatology, Director, Fellowship Program in Neonatal-Perinatal Medicine, Director, Transport/ECMO/Nutrition, Vice Chair, Clinical Research, Department of Pediatrics, Children's Hospital of Georgia

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

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Gerber.

Additional Contributors

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, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

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