Serial transverse enteroplasty procedure (STEP) for bowel lengthening in parenteral nutrition-dependent children (interventional procedures consultation)
NATIONAL INSTITUTE FOR HEALTH AND CLINICAL EXCELLENCE
Interventional Procedure Consultation Document
Serial transverse enteroplasty procedure (STEP) for bowel lengthening in parenteral nutrition-dependent children
|In children, some diseases may result in abnormally short bowel. This can cause severe nutritional problems because of insufficient food absorption. Serial transverse enteroplasty is a procedure where the bowel is cut and stapled in a zigzag pattern in order to narrow and lengthen it.|
The National Institute for Health and Clinical Excellence is examining serial transverse enteroplasty procedure (STEP) for bowel lengthening in parenteral nutrition-dependent children and will publish guidance on its safety and efficacy to the NHS in England, Wales, Scotland and Northern Ireland. The Institute's Interventional Procedures Advisory Committee has considered the available evidence and the views of Specialist Advisers, who are consultants with knowledge of the procedure. The Advisory Committee has made provisional recommendations about serial transverse enteroplasty procedure (STEP) for bowel lengthening in parenteral nutrition-dependent children.
Note that this document is not the Institute's formal guidance on this procedure. The recommendations are provisional and may change after consultation.
The process that the Institute will follow after the consultation period ends is as follows.
For further details, see the Interventional Procedures Programme manual, which is available from the Institute's website (www.nice.org.uk/ipprogrammemanual).
Closing date for comments: 24 June 2007
|Note that this document is not the Institute's guidance on this procedure. The recommendations are provisional and may change after consultation.|
|1.1||The evidence on safety of the serial transverse enteroplasty procedure (STEP) for bowel lengthening in parenteral nutrition dependent children is adequate; however, there is limited evidence of efficacy. Therefore, this procedure should not be used without special arrangements for consent and for audit or research.|
Clinicians wishing to undertake STEP for bowel lengthening in parenteral nutrition-dependent children should take the following actions:
|1.3||Patient selection should be carried out in the context of a multidisciplinary team experienced in the management of short bowel syndrome.|
|1.4||Clinicians undertaking STEP for bowel lengthening in parenteral nutrition-dependent children should submit data on all patients to the International STEP Data Registry at Children's Hospital Boston, USA (available from www.childrenshospital.org/cfapps/step/index.cfm).|
|2.1.1||Short bowel syndrome (SBS) is a rare but serious condition which may be congenital or iatrogenic. Patients with SBS have a rapid intestinal transit time which results in malabsorption of enteral nutrition and subsequent development of malnutrition. Most patients with SBS require partial or total parenteral nutrition (TPN). TPN can cause liver failure and recurrent sepsis leading to death.|
|2.1.2||The causes of SBS can be either structural, (short jejunoileal length) or functional (damaged or dysfunctional intestinal mucosa). Conditions associated with SBS include jejunoileal atresia, gastroschisis or omphalocele, Hirschsprung's disease, necrotising enterocolitis, or intestinal volvulus. All but the latter present in the neonatal period.|
|2.1.3||Surgical procedures which prolong bowel transit time and aim to increase the absorption of nutrients include resection of dilated segments of the small intestine, tapering enteroplasty, intestinal placation, or the Bianchi intestinal loop lengthening procedure. Some patients require small bowel and liver transplants.|
|2.2||Outline of the procedure|
|2.2.1||STEP is a surgical technique that aims to lengthen the small intestine of patients with SBS so that they can benefit from enteral nutrition.|
|2.2.2||The procedure relies on the principle that the blood supply to the small bowel originates from the mesentery and traverses the bowel perpendicular to its long axis. Under general anaesthesia, an endoscopic stapler is passed through the mesentery and the bowel is simultaneously stapled and dissected on alternating sides in a direction perpendicular to its long axis. The small bowel is left with a zigzag appearance.|
|2.3.1||A case series of 38 patients (29 with SBS) reported an increase in mean bowel length from 68 cm at baseline to 115 cm (p < 0.0001) in 27 patients where this outcome was measured. A second case series of five patients showed an increase in bowel length from 61 cm at baseline to 98 cm immediately following the procedure (p < 0.01). A third case series of eight patients, with five patients undergoing STEP, reported an increase in mean bowel length of 17 cm, from an average of 62 cm to 79 cm. A case report of a patient undergoing STEP for SBS and D-lactic acidosis reported that a normal bowel transit time (2.5 hours) was achieved at 7 days' follow-up.|
|2.3.2||In the case series of 38 patients, 21 of whom were on TPN at baseline, there was a mean improvement from 31% at baseline to 67% (p < 0.01) in the total percentage of calories tolerated enterally at 13 months' follow-up. Of these 21 patients, 10 were completely weaned off TPN (follow-up not stated). In the case series of five patients, it was reported that the mean percentage of nutrition received enterally improved from 49% at baseline to 80% in three patients followed up at 17 months (p < 0.05). The case series of eight patients reported that, of the five undergoing STEP as monotherapy, 'more than 50%' were completely weaned off TPN and one patient had significantly decreased dependency (not otherwise defined). In two case reports, STEP allowed for 75% and 100% of calorific intake to be achieved enterally at 11 and 7 months, respectively. For more details, refer to the sources of evidence (see appendix).|
|2.3.3||One Specialist Adviser commented that STEP is one of a range of surgical options, none of which has been shown convincingly to provide benefit. Most of the Specialist Advisers suggested that the prognosis of SBS is uncertain and that some patients improve spontaneously without treatment.|
|2.4.1||In one case series with 13 months' follow-up, the mortality rate following STEP was 8% (3/38) and three patients (8%) went on to require a bowel or liver and bowel transplant. One patient in a case series of eight developed cholestasis which caused sepsis, liver failure, and subsequently death at 3 months.|
|2.4.2||A case series of five patients and a case report of one patient reported no long-term complications at 15 and 7 months' follow-up, respectively.|
|2.4.3||A patient in a case report required nasogastric fluid aspiration postoperatively. This was bilious at first, becoming clear over time. For more details, refer to the sources of evidence (see appendix).|
|2.4.4||The Specialist Advisers considered this procedure to be a simple operation with low morbidity. The Specialist Advisers considered the potential risks associated with this procedure to include staple-line leak, bowel obstruction, pleural effusion, hypertension, haematoma, abscess formation, mortality and progression to transplant. They stated that other theoretical complications include further bowel shortening, fistula formation, bleeding, deteriorating bowel or liver function, cholestasis and septic complications.|
Chairman, Interventional Procedures Advisory Committee
|Appendix:||Sources of evidence|
The following document, which summarises the evidence, was considered by the Interventional Procedures Advisory Committee when making its provisional recommendations.
Available from: www.nice.org.uk/ip385overview.
This page was last updated: 30 March 2010