Gastroenterology

Gastroenterology

Volume 130, Issue 2, Supplement, February 2006, Pages S138-S146
Gastroenterology

Original Article
From the Cradle to Enteral Autonomy: The Role of Autologous Gastrointestinal Reconstruction

https://doi.org/10.1053/j.gastro.2005.09.070Get rights and content

The short bowel state is treatable, with acceptable long-term quality of life. Management during the first 6–12 months of life is critical but, presently, frequently compromises long-term survival and prospects. At first presentation, primary caregivers, working with specialists at designated intestinal failure centers, should develop a structured individual-oriented management plan. Preservation of venous access, “hepatosparing” parenteral nutrition, and avoidance of liver sepsis are crucial to survival. Early surgery should be limited to conservation of autologous bowel, even short bowel lengths having great potential, and to facilitating natural intestinal adaptation. Bowel expansion may be relevant prior to delayed bowel reconstruction with single or combined techniques that include bowel lengthening and/or tailoring, reversed segments, and colon interposition. Bowel transplantation, as yet not recommended for primary management, offers survival and opportunity to those with no prospect of autologous bowel autonomy or following failed autologous gastrointestinal reconstruction. This paper reviews current surgery for the short bowel state and concludes that it is presently appropriate before bowel transplantation to offer autologous gastrointestinal reconstruction, with its prospect of enteral autonomy with quality life. It emphasizes the need for an individual-oriented management plan, developed jointly at the time of first presentation between the primary caregivers and a designated multidisciplinary intestinal failure center, to enhance the prospects for enteral autonomy preferably on autologous bowel. Autologous gastrointestinal reconstruction is in its infancy and requires resources, commitment, and research from dedicated bowel reconstructive surgeons toward a better opportunity for the child and family with short bowel.

Section snippets

The Short Gut Problem

Antenatal midgut loss presents with bowel atresia and intestinal obstruction. The massively dilated residual proximal small bowel represents some 10%–20% of normal small bowel length, with the distal colon often being the next available bowel. In this context, the natural doubling in bowel length during the last trimester of pregnancy is an advantage to the premature child.1 Attempted relief of mechanical bowel obstruction by anastomosis of the massively dilated proximal jejunum to the small

PN

It must be acknowledged that PN has been the key factor in survival for the short gut patient. Its administration is complex, and infection hazards are constant. Detailed analysis of PN is beyond the scope of this paper. It is, however, relevant to note that the feeding solutions are not perfect and are thought to be partly responsible for severe cholestasis with hepatic fibrosis rapidly leading to end-stage liver failure and a reduced bowel adaptive response, impacting on the timely

AGIR Techniques

Surgical attempts at improving mucosal absorption have been varied and imaginative (Table 2). Some, such as “recycling loops” and “pouches,” have not proven beneficial. Others, including bowel pacing, neomucosal growth, mucosal stem cell regeneration, and tissue-engineered intestine,7 may yet develop to clinical application. Progressive understanding of bowel physiology has stimulated varied and interesting concepts, which have been excellently reviewed by Thompson and Rikkers (1987),8 Warner

Common Trends in AGIR Techniques

Analysis of the various techniques in AGIR reveals some common trends. Most procedures are relatively simple and free from major morbidity or mortality, and some, such as colon interposition, reversed segments, and intestinal valves are reversible. All have been shown to slow transit, to increase mucosal contact, and to enhance absorption. The LILT procedure has been associated with significant increases in absorption, allowing many patients to be weaned from PN. It is abundantly clear from the

Criteria for AGIR and Transplantation

Barksdale and Stanford10 suggested that the indications for AGIR possibly within the first year of life are a failure to progress with weaning from PN and the presence of life-threatening complications such as PN-related liver disease, recurrent central line sepsis, and limited vascular access. The authors indicate that patients with ultrashort segment disease, ie, <10% with ICv, or <20% without ICv, often benefit from “adjunctive proadaptive surgery,” ie, AGIR with the choice of procedure

Discussion

The management of the child with a short bowel is a complex multidisciplinary process over a prolonged period of several years. The long-term outlook for these children continues to improve with developments in PN support, bowel reconstructive procedures, and transplantation. The number of children with short bowel and intestinal failure is not great, and early management has often been uncoordinated, haphazard, and less than ideal. Current evidence can only be indicative of potential benefits

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