|Year : 2023 | Volume
| Issue : 2 | Page : 103-110
Intestinal Bowel Lengthening within the First 6 Months of Life: Institutional Experience and Review of the Literature
Andrea Zulli1, Riccardo Coletta1, Bashar Aldeiri2, Antonino Morabito1
1 Department of Pediatric Surgery, Meyer Children's Hospital, University of Florence, Florence, Italy
2 Department of Pediatric Surgery, Royal Manchester Children's Hospital, Manchester, United Kingdom
|Date of Submission||28-Sep-2021|
|Date of Decision||12-Mar-2022|
|Date of Acceptance||26-Mar-2022|
|Date of Web Publication||03-Mar-2023|
Department of Pediatric Surgery, Meyer Children's Hospital, University of Florence, Viale G. Pieraccini 24, 50139 Florence
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Background: Management of short bowel syndrome in children has been surrounded by much debate with timing of the lengthening procedure still controversial. Early bowel lengthening procedure (EBLP) has been defined as any bowel lengthening procedure performed before 6 months of age. The purpose of this paper is to report the institutional experience in EBLP and to review the literature on this subject to identify common indications.
Methods: An institutional retrospective analysis of all the intestinal lengthening procedures was performed. Furthermore, an Ovid/Embase search regarding children who underwent bowel lengthening in the past 38 years was conducted. Primary diagnosis, age at procedure, type of procedure, indication, and outcome were analyzed.
Results: Ten EBLP were performed in Manchester from 2006 to 2017. Median age at surgery was 121 days (102–140), preoperative small bowel (SB) length was 30 cm (20–49) while postoperative SB length was 54 cm (40–70), with a median increased bowel length of 80%. Ninety-seven papers were reviewed, with more than 399 lengthening procedures performed. Twenty-nine papers matched criteria with more than 60 EBLP were observed of which 10 were performed in a single center from 2006 to 2017. EBLP was performed due to SB atresia, to excessive bowel dilatation or failure to enteral feeds, at a median age of 60 days (1–90). Serial transverse enteroplasty was the most frequent procedure used lengthening the bowel from 40 cm (29–62.5) to 63 cm (49–85), with a median increased bowel length of 57%.
Conclusions: This study confirms that no clear consensus on indication or timing to perform early SB lengthening is reported. According to the gathered data, EBLP should be considered, only in cases of actual necessity after review of qualified intestinal failure center.
Keywords: Autologous gastrointestinal reconstructive surgery, bowel lengthening, children, serial transverse enteroplasty, short bowel syndrome
|How to cite this article:|
Zulli A, Coletta R, Aldeiri B, Morabito A. Intestinal Bowel Lengthening within the First 6 Months of Life: Institutional Experience and Review of the Literature. J Indian Assoc Pediatr Surg 2023;28:103-10
|How to cite this URL:|
Zulli A, Coletta R, Aldeiri B, Morabito A. Intestinal Bowel Lengthening within the First 6 Months of Life: Institutional Experience and Review of the Literature. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Jun 2];28:103-10. Available from: https://www.jiaps.com/text.asp?2023/28/2/103/371171
| Introduction|| |
Short bowel syndrome (SBS) is a severe multisystemic disorder that results from the loss of a significant amount of small bowel (SB). The incidence of severe SBS is estimated to be 25/100,000 live births, most sufferers are infants and young children.
The debate about the correct definition of SBS is still open. Bowel length of <100 cm seems to be abnormal, but according to different authors, generally <40 cm requires therapy. The most frequent causes of SBS are necrotizing enterocolitis (NEC), small intestinal volvulus in intestinal malrotation, gastroschisis, and SB atresia.
The management of SBS has evolved tremendously over the past two decades, improving patients' survival rates. This has mainly been due to surgical techniques and improvements in total parenteral nutrition (TPN). However, complications of TPN still represent a major challenge to pediatric intestinal rehabilitation teams.
Different bowel lengthening techniques have been used in recent years, often causing much controversy with varying results. Type of surgery is case specific and remaining bowel length and degree of bowel distension are important factors to consider when choosing the best procedure for the patient.
The most frequently chosen autologous gastrointestinal reconstructions (AGIRs) are longitudinal intestinal lengthening and tailoring (LILT), proposed by Bianchi in 1980 and serial transverse enteroplasty (STEP) proposed by Kim in 2003. More recently, the Spiral Intestinal Lengthening and Tailoring technique (SILT) has been introduced, which allows lengthening of an intestinal segment with a lesser degree of bowel dilatation. Others techniques, such as antiperistaltic reverse segment or the more recently introduced transverse flap duodenoplasty, can be applied in combinations with the older techniques. A structured pathway for managing these patients was proposed by Manchester Children's Hospital team in 2012. However, the timing of intervention in patients with SBS is still debated.
The purpose of this review of literature about early SB lengthening (elongation procedures performed before 6 months of life) is to examine the outcomes and combining it with our intestinal rehabilitation unit experience.
| Methods|| |
Children with SBS treated at the Royal Manchester Children's Hospital between 2006 and 2017 were analyzed.
An Ovid/Embase search for all the children who underwent bowel lengthening procedures was performed. Research terms were SBS, intestine lengthening, human, children. Review papers and non-English papers were excluded from this review.
We analyzed the type of paper, number of cases, number of early lengthening cases, primary diagnosis, and reason for early SB lengthening. We observed the age at procedure, SB length before lengthening, type of the procedure, primary diagnosis, SB length achieved, number of cases in which parenteral nutrition (TPN) was stopped and TPN duration, number of deceased patients, and complications after surgery.
Data are presented as median ± interquartile range. This study was conducted in accordance with the International Conference for Harmonization Good Clinical Practice and with the current revision of the Declaration of Helsinki. The study was exempted by the local institutional review board.
| Results|| |
Between 2006 and 2017, of the 36 SBS procedures performed at the Royal Manchester Children's Hospital, 10 were performed early; four infants were males and six were females. The median gestation at birth was 34 weeks (range: 32.5–34 weeks), and the median age at surgery was 121 days (range: 102–140 days). The primary diagnoses of SBS were six gastroschisis, two NEC, one Atresia with volvulus, and one multiple atresia.
Indications for early lengthening were re-dilatation following primary anastomosis, failure of enteral nutrition, and allowing for natural SB growth. Survival rate was 90%; none of the patients required repeated lengthening. Four reached full enteral autonomy and four needed partial home parenteral nutrition. The breakdown of the type of lengthening procedures performed at the hospital was three LILT, three STEP, two SILT, one LILT and STEP, and one STEP and reverse interposition. Preoperative SB length was 30 cm (range: 20–49 cm), while postoperative SB length was 54 cm (range: 40–70 cm), with a median increased bowel length of 80%.
Ninety seven papers were reviewed that mentioned 399 lengthening procedures performed over a 38-year period. Twenty-nine papers matched our criteria, including 18 single-center studies and 11 case reports. More than 60 early short bowel lengthening procedures were recorded [Flow Diagram 1].
The main identified cause of SBS in these patients was SB atresia (26 cases); the other causes included gastroschisis (n = 6), midgut volvulus (n = 6), SB atresia + gastroschisis (n = 5), internal hernia (n = 1), and intestinal ischemia (n = 1). Causes could not be ascertained in 14 cases.
The AGIR procedures reported were included STEP (n = 35), LILT (n = 20), anterior flap (n = 4), and duodenal flap (n = 1). The main reason for the early lengthening procedure was excessive dilatation due to SB atresia (22 cases), followed by failure of enteral feeding (n = 3), failure to enteral feeding with Sepsis (n = 1), failure to enteral feeding with bowel dilatation (n = 1), high stool output (n = 5), and a very short bowel (n = 1). The clinical indication to perform an early bowel lengthening procedure could not be ascertained in 25 patients.
Median age at lengthening was 60 days (range: 1–90 days). Median initial SB length was 40 cm (range: 29–62.5 cm), and median length achieved was 63 cm (range: 49–85 cm), with a median increased bowel length of 57%. Parenteral nutrition was stopped after a median time of 180 days (range: 90–247.5 days). Fifteen patients (25%) weaned off PN [Table 1]. These data were compared with literature review [Table 2].
Complications reported were sepsis (n = 19), excessive dilatation requiring re-STEP procedure (n = 15), intestinal failure associated liver disease (n = 3), bowel obstruction (n = 3), enterocutaneous fistulas (n = 2), cholestasis (n = 1), low body weight after procedure (n = 1), anastomosis leakage (n = 1), and transaminase elevation (n = 1). Eight patients underwent intestinal transplantation while one required liver transplantation. Fifteen percent of patients died due to liver failure (n = 9). Other causes of death included “SBS complications” not specified (n = 2), sepsis with liver failure (n = 1), sepsis (n = 1), heart disease (n = 1), and upper gastrointestinal hemorrhage (n = 1).
| Discussion|| |
Current management of children with SBS is known to be a complex, prolonged process where the pediatric surgeon is constantly facing difficult challenges to overtake all the nutritional and social problems related to this condition. Several studies have demonstrated that multidisciplinary approach to SBS has improved dramatically the surgical and medical outcomes of this patients.,
In modern intestinal rehabilitation centers, these patients can find the most update options to achieve the best possible results such as unstructured pathway that includes medical, surgical, and psychological support, which are tailored to individual patient needs., Reconstructive surgery can involve one or more procedures, such as LILT, SILT, or STEP technique. These procedures seem to promote intestinal function by increasing the intestinal absorptive surface and slowing bowel transit.,
Despite no consensus about the correct timing to perform lengthening procedure has been stated, the remaining bowel length and the degree of bowel dilatation are the two recognized characteristics, which can suggest the need of performance of the procedure. Shorter intestinal length has been associated with poor outcome in terms of survival and adaptation., The presence of the ileocecal valve and the colon has been considered an important factor by some authors,, while it was not considered important to parenteral nutrition (PN) weaning in other studies.,, All aspects of although SBS could be performed at any age, it was suggested that early surgical intervention had advantage of normal growth and development. Some authors suggested a benefit of earlier adaptation when AGIR surgery is offered during the first 1 year of life, while others recommended surgical intervention only after a period of bowel adaptation.
In fact, many factors have been taken into consideration to determine what is responsible for better outcomes in patients with SBS. Some studies suggest that outcomes are worse in patients with SBS due to complex gastroschisis,, while others did not find a correlation between etiology and survival or ability to achieve enteral autonomy.,,,
In case of un-dilated bowel, we can wait for a period of 20–24 weeks of bowel before performing an AGIR procedure., A tube stoma system can be used: a catheter is placed in the proximal SB distal end secured using a purse-string suture and it is sutured to the anterior abdominal wall. The catheter is then brought out of the abdominal wall. The distal bowel is treated in the same way. The child is fed postoperatively, clamping the proximal tube stoma for progressively longer periods to allow bowel expansion. Fecal recirculation is then performed: the effluent of the proximal tube stoma is slowly recycled into a similar tube stoma in the distal bowel to aid absorption. Oral nutrition is generally preferred to stimulate the swallowing mechanism and to avoid food aversion during this period.
Review of literature also shows that the early intestinal lengthening procedures have higher rates of postoperative complications and re-intervention, with less than optimal rates of intestinal stretch as compared to our Manchester experience, but obviously it would be incorrect to compare a historical cohort of patients with a group of patients who underwent surgery in more recent times [Table 2].
Moreover, more patients needed parenteral than recent analyses, with a similar PN duration.,,,,,, In fact, only fifteen patients (25%) could be weaned off PN and achieved enteral autonomy. In those patients who were weaned off PN, the time to achieve enteral autonomy varied widely (30–900 days), with a duration of PN comparable to that of the previous studies., The elevated number of deaths in these studies could be reflective of unreported comorbidity of these patients and as well as lacking clinical data in the articles.
LILT and STEP procedures are the most popular surgical techniques used for AGIR, while they remain technically challenging and not the best option in mildly dilated SB (2–5 cm). The study confirmed that LILT and STEP successfully elongated the bowel up to 100% and 75%, respectively; however, STEP procedure generally did not allow other AGIR procedures to be easily performed.
Initially, in Manchester experience, LILT procedures were mainly carried out. Later, the introduction of other techniques such as STEP has allowed to broaden the therapeutic options and to be able to combine different surgical procedures. The advent of new techniques such as SILT and duodenal flap will probably contribute to further modifying these percentages and outcomes. The patients who underwent surgery in Manchester were older and had better outcomes, probably due to both the timing of intervention and to better team experience in managing these patients.
Till some years ago, early structured intervention was generally known to improve the clinical outcome in infants with SBS by reducing the morbidity and mortality associated with PN; our data seems to be in agreement with some other authors who recommended surgical intervention only after a period of bowel adaptation, as the natural process may obviate the need for any other interventions. Outcomes mostly depend on the ability of the residual gastrointestinal tract to adapt functionally.
This literature review showed that high rate of re-STEP procedures and sepsis episodes occurs in the postoperative period. It can be justified by an excessive rapid bowel dilatation with a consequent bacterial overgrowth. This involves a subsequent second surgery in the postoperative period, with a high anesthesiological and surgical risks for the infant and not allowing a gradual bowel adaptation, a main goal in SBS patients.
A more aggressive approach with surgery allowed earlier reduction of PN nutrition and bowel lengthening with good results but these results seem to be worse when compared with recent bowel lengthening studies. The number of patients reviewed in our study, however, is limited, and it was not possible to recover all the data concerning the various parameters for all the 60 reviewed patients.
| Conclusions|| |
SBS patients are difficult to treat from the 1st days of life. While previously there was the belief that we had to intervene aggressively as quickly as possible to allow better intestinal adaptation, our review found high rate of complications and a difficulty to wean off PN. Previously, the main indication for intervention was the impossibility of feeding these patients enterally but progress in the production of specific parenteral formulations made it possible to reduce surgical procedures. Therefore, we believe that early lengthening procedures should be considered only in cases of actual necessity.
A multidisciplinary approach, with gastroenterologists, pediatric surgeons, nutritionists, radiologists, anesthesiologists, and other figures, remains important in improving the management and short- and long-term results of these patients.
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Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2]