Epidemiology, management and outcome of ultrashort bowel syndrome in infancy

Abstract

Ultrashort bowel syndrome (USBS) is a group of heterogeneous disorders where the length of small bowel is less than 10 cm or 10% of expected for the age. It is caused by massive loss of the gut which in the neonatal period can be a result of vanishing gastroschisis or surgical resection following mid-gut volvulus, jejunoileal atresia and/or extensive necrotising enterocolitis. The exact prevalence of USBS is not known although there is a clear trend towards increasing numbers because of increased incidence and improved survival. Long-term parenteral nutrition (PN) is the mainstay of treatment and is best delivered by a multidisciplinary intestinal rehabilitation team. Promoting adaptation is vital to improving long-term survival and can be achieved by optimising feeds, reducing intestinal failure liver disease and catheter-related bloodstream infections. Surgical techniques that can promote enteral tolerance and hence improve outcome include establishing intestinal continuity and bowel lengthening procedures. The outcome for USBS is similar to patients with intestinal failure due to other causes and only a small proportion of children who develop irreversible complications of PN and will need intestinal transplantation. In this review, we will summarise the available evidence focusing particularly on the epidemiology, management strategies and outcome.

Intestinal failure: the evolving demographic and patient outcomes on home parenteral nutrition

“…This escalation can be attributed to various factors including the improved survival of extremely preterm infants at risk of developing NEC, the increased incidence of gastroschisis , the reduced mortality in children with IF and a reduction in the number of children requiring small bowel transplant because of complications of PN . In addition to this, there has been an enhanced confidence in using long‐term PN in children with severe forms of irreversible IF, such as USBS and MMIH . There has also been a change in the demographics of patients managed at home.…”

Nutritional management of surgical newborns and infants in the acute phase and during follow up

“…12 If human milk is not available, there is a lack of consensus on whether a hydrolysed formula, either as extensively or partially hydrolysed formula, should be used as a first choice rather than intact cow's milk formula. 12,22,23 It has also to be taken into consideration that the use of hydrolysed formulas has been associated with faster gastric emptying and intestinal transit time, which may not be beneficial in infants who have undergone bowel length reduction. On the other hand, hydrolysed formulas have been reported to promote more efficient protein digestion, even though available data are not conclusive.…”

“…Fat intake greatly contributes to better growth; moreover, fatty acids metabolized to short chains appear to promote gastrointestinal adaptation and functions. 12,22,23 Enteral nutrition may be delivered through bolus feeding, continuous feeding or a combination of both. Bolus feeding mimics a physiological situation and promotes gastrointestinal motility, enterohepatic circulation of bile acids, gall bladder contraction and pulsatile release of gastrointestinal hormones.…”

“…However, it must be taken into account that, in addition to the reduction of the intestinal length, impaired intestinal function may coexist, further contributing to inadequate absorption of enteral nutrients. 11,12,23,35 In Table 1 most common causes of short bowel syndrome in newborns and infants are indicated. The frequency of surgical short bowel syndrome was reported to be 0.7% among very low birth weight infants born during the period from 2002 to 2005 at the National Institute of Child Health and Development Neonatal Research Network Centres, and it showed an inverse relationship with birth weight.…”

“…The time interval necessary to reach the maintenance phase is variable depending on the infant's clinical conditions and complications. 12,23 …”