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Short Bowel Syndrome 427

Isolated liver transplants can be offered to some infants with early-
A B onset liver failure but with sufficient bowel length such that adaptation
could be expected.
The 5-year survival is approximately 70%; however, around 25% of
patients die while on the waiting list for a transplant.
Prognosis and Outcomes of SBS
Long-term survival without TPN depends on the remaining bowel
length. With TPN availability, survival is related to complications of
TPN rather than to bowel length. The overall mortality of infants with
SBS on TPN is 10–25%.
The two main causes of death and long-term morbidity in patients
with short gut on TPN are liver failure and sepsis. In children, liver
failure is secondary to intrahepatic cholestasis. This is most common
in those who are entirely TPN dependent with no enteral feeding. It
is also seen more frequently in neonates who are premature, have
Figure 70.3: The STEP procedure: (A) A GIA stapler is used transversely low birth weight, and have recurrent sepsis. The other main cause of
across the dilated bowel from the antimesenteric border, leaving 2 cm of death—septicaemia—arises because a complete lack of enteral nutrition
the bowel diameter uncut. The next cut is taken too distally this time from results in bacterial overgrowth of the bowel and mucosal atrophy with
the antimesenteric border via a small gap created in the mesentery. (B) The
GIA stapler is used down the bowel, alternating between the mesenteric and impaired mucosal immunity, leading to an increased incidence of sepsis.
antimesenteric border as shown (dotted lines). Care is taken to keep the GIA Recurrent septicaemia is also related to central venous catheters. Early
stapler perpendicular to the mesentery to preserve the blood supply. enteral feeding is therefore vital if these complications are to be reduced.
Even after discharge from hospital on full enteral feeds, infants
are at risk during episodes of enteric infection, when rapid fluid and
The STEP procedure is shown in Figure 71.3. STEP registry figures electrolyte loss may require emergency admission for intravenous
show a mean pre-STEP bowel length of 68 cm achieving a mean post- rehydration. The management of a patient with SBS requires a
STEP bowel length of 115 cm. The percentage of enteral feeding increased multidisciplinary team, including paediatrician, surgeon, community
from a mean of 33% preoperatively to a mean of 63% postoperatively. nurse, dietitian, and pharmacist.
Complications of bowel-lengthening procedures are high, including An audit of 63 patients with SBS seen at the Red Cross Children’s
anastomotic and staple line leaks, bowel obstruction from adhesions or Hospital between 1998 and 2006 revealed the following: The mean
ischaemic strictures, bleeding, abscess formation, and death. gestational age was 32 weeks (range 25–40 weeks). The most
The limitations of bowel-lengthening procedures have led some frequent causes were necrotising enterocolitis (NEC) (40%), along
authors to advocate that they should be reserved for those patients who, with intestinal atresia, midgut volvulus, intestinal aganglionosis, and
after 6 months of bowel adaptation, are tolerating more than half of their gastroschisis. Overall, mortality was 36.5% (23/63). The mean number
feeds enterally and would therefore have a greater chance of successfully of days on parenteral nutrition was 95 (range 30–420 days).
becoming fully enterally fed following a lengthening procedure.
Intestinal Transplant Ethical Issues
Intestinal transplant is offered in only a few centres worldwide. It is not The annual cost of care of a patient with SBS on parenteral nutrition has
an alternative to long-term TPN. It is reserved only for patients who been estimated at between $100,000 and $150,000, making such care
are unable to have TPN, usually due to TPN-related liver disease or beyond the reach of all but a few. Treatment of patients with significant
difficulty with venous access for TPN administration. bowel loss in resource-poor settings is likely to be limited to those who
Intestinal transplant may involve (1) isolated bowel, for those with attain enteral feeding quickly and have sufficient bowel function to
good liver function and normal motility; (2) bowel plus liver, for those require only increased oral calories and vitamin and mineral supple-
with liver disease; or (3) multivisceral, which includes liver, bowel, mentation. There is therefore a need to counsel parents before surgery
stomach, and pancreas, for those with multiple abdominal organ failure for bowel conditions that can potentially lead to short gut syndrome.
and dysmotile bowel. The most frequent transplant performed for Evidence-Based Research
children with SBS is a liver plus bowel transplant. This procedure is Table 71.2 presents a comparison of intestinal-lengthening procedures
currently limited to children weighing more than 5 kg due to the lack of for patients with SBS.
size-matched donors.