Overview of Pediatric Short Bowel Syndrome

Division of Gastroenterology and Nutrition and Center for Advanced Intestinal Rehabilitation at Children's Hospital Boston, Harvard Medical School, Boston, MA 02115, USA.
Journal of pediatric gastroenterology and nutrition (Impact Factor: 2.63). 09/2008; 47 Suppl 1(Suppl 1):S33-6. DOI: 10.1097/MPG.0b013e3181819007
Source: PubMed


Short bowel syndrome (SBS) is a malabsorptive state occuring as a result of surgical resection or congenital disease of a significant portion of the small intestine . The amount of resection or remaining bowel generally dictates the degree of malabsorption and consequentely the need for specialized enteral nutrition or parenteral nutrition (PN). Intestinal failure in the context of SBS is defined as a dependence on PN to maintain minimal energy and fluid requirement for growth in children. Common causes of SBS in infants and children include necrotizing enterocolitis, midgut volvulus, intestinal atresia, and gastroschisis. Early identification of patients at risk for long-term PN dependency is the first step toward avoiding severe complications. Close monitoring of nutritional status, steady and early introduction of enteral nutrition, and aggressive prevention, diagnosis, and treatment of infections such as central venous catheter sepsis and bacterial overgrowth can significantly improve the prognosis. Intestinal transplantation is an emerging treatment that may be considered when intestinal failure is irreversible and children are experiencing serious complications related to TPN administration.

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    • "SBS is a highly morbid disease, characterized by septic episodes, metabolic derangements, repeated hospitalizations, and a poor quality of life [6]. The care of children with SBS remains challenging, requiring meticulous management of PN and vigilance with regards to infectious complications [1] [7]. Counseling these patients and their families is made difficult by the relatively unpredictable course of the disease. "
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    ABSTRACT: In a large cohort of children with intestinal failure (IF), we sought to determine the cumulative incidence of achieving enteral autonomy and identify patient and institutional characteristics associated with enteral autonomy. A multicenter, retrospective cohort analysis from the Pediatric Intestinal Failure Consortium was performed. IF was defined as severe congenital or acquired gastrointestinal diseases during infancy with dependence on parenteral nutrition (PN) >60 days. Enteral autonomy was defined as PN discontinuation >3 months. A total of 272 infants were followed for a median (IQR) of 33.5 (16.2-51.5) months. Enteral autonomy was achieved in 118 (43%); 36 (13%) remained PN dependent and 118 (43%) patients died or underwent transplantation. Multivariable analysis identified necrotizing enterocolitis (NEC; OR 2.42, 95% CI 1.33-4.47), care at an IF site without an associated intestinal transplantation program (OR 2.73, 95% CI 1.56-4.78), and an intact ileocecal valve (OR 2.80, 95% CI 1.63-4.83) as independent risk factors for enteral autonomy. A second model (n = 144) that included only patients with intraoperatively measured residual small bowel length found NEC (OR 3.44, 95% CI 1.36-8.71), care at a nonintestinal transplantation center (OR 6.56, 95% CI 2.53-16.98), and residual small bowel length (OR 1.04 cm, 95% CI 1.02-1.06 cm) to be independently associated with enteral autonomy. A substantial proportion of infants with IF can achieve enteral autonomy. Underlying NEC, preserved ileocecal valve, and longer bowel length are associated with achieving enteral autonomy. It is likely that variations in institutional practices and referral patterns also affect outcomes in children with IF. Copyright © 2015 Elsevier Inc. All rights reserved.
    2014 American Academy of Pediatrics National Conference and Exhibition; 10/2014
    • "Secondary outcomes measured were the inter-center variation in LOS, the duration of parenteral nutrition (PN), the duration of prophylactic anti-microbial treatment, the rates of central-line utilization and associated blood stream infections (CLABSI) [17]. The prevalence of necrotizing enterocolitis (NEC) [18] and short bowel syndrome/intestinal failure (SBS/IF) that became evident after closure [19] was also ascertained. SBS/IF was defined when infants had a major bowel resection such that b50% of the expected length or b75 cm of small bowel remained post-operatively [20] and with the subsequent receipt of ≥ 90 days of PN [21] [22] [23]. "
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    ABSTRACT: Background/Purpose The optimal surgical approach in infants with gastroschisis (GS) is unknown. The purpose of this study was to estimate the association between staged closure and length of stay (LOS) in infants with GS. Design/Methods We used the Children's Hospital Neonatal Database to identify surviving infants with GS born ≥ 34 weeks' gestation referred to participating NICUs. Infants with complex GS, bowel atresia, or referred after 2 days of age were excluded. The primary outcome was LOS; multivariable linear regression was used to quantify the relationship between staged closure and LOS. Results Among 442 eligible infants, staged closure occurred in 68.1% and was associated with an increased median LOS relative to primary closure (37 vs. 28 days, p < 0.001). This association persisted in the multivariable equation (β = 1.35, 95% CI: 1.21, 1.52, p < 0.001) after adjusting for the presence of necrotizing enterocolitis, short bowel syndrome, and central-line associated bloodstream infections. Conclusions In this large, multicenter cohort of infants with GS, staged closure was independently associated with increased LOS. These data can be used to enhance antenatal and pre-operative counseling and also suggest that some infants who receive staged closure may benefit from primary repair.
    Journal of Pediatric Surgery 08/2014; 49(8). DOI:10.1016/j.jpedsurg.2013.12.020 · 1.39 Impact Factor
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    • "SBS is a malabsorptive state that may follow massive resection of the small intestine because the remaining small bowel absorptive surface is inadequate and enteral nutrient intake cannot support the body mass4, 5). SBS in children usually results from necrotizing enterocolitis and congenital intestinal anomalies, for which surgical resection of the involved intestinal segment is often the only treatment, as exemplified in our patient6). "
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    ABSTRACT: Anemia caused by vitamin B12 deficiency resulting from inadequate dietary intake is rare in children in the modern era because of improvements in nutritional status. However, such anemia can be caused by decreased ingestion or impaired absorption and/or utilization of vitamin B12. We report the case of an 18-year-old man with short stature, prepubertal sexual maturation, exertional dyspnea, and severe anemia with a hemoglobin level of 3.3 g/dL. He had a history of small bowel resection from 50 cm below the Treitz ligament to 5 cm above the ileocecal valve necessitated by midgut volvulus in the neonatal period. Laboratory tests showed deficiencies of both vitamin B12 and iron. A bone marrow examination revealed dyserythropoiesis and low levels of hemosiderin particles, and a cytogenetic study disclosed a normal karyotype. After treatment with parenteral vitamin B12 and elemental iron, both anemia and growth showed gradual improvement. This is a rare case that presented with short stature and delayed puberty caused by nutritional deficiency anemia in Korea.
    Korean Journal of Pediatrics 05/2010; 53(5):661-5. DOI:10.3345/kjp.2010.53.5.661
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