The 2 STEP: an approach to repeating a serial transverse enteroplasty

Purpose

The serial transverse enteroplasty procedure (STEP) procedure is a successful and safe approach to lengthen small bowel in patients with short bowel syndrome (SBS). However, postlengthening dilatation may occur, which can lead to bacterial overgrowth and malabsorption. We addressed this problem by reperforming the STEP in 2 patients.

Methods

Two infants underwent the STEP procedure at 3 days and at 4 months of life for SBS secondary to intestinal atresia and gastroschisis. The patients' small bowel lengths were 20 and 32 cm before and 25 and 52 cm after the initial STEP. Nine and 8.5 months afterward, the patients developed dilatation of the small bowel and feeding intolerance. A second STEP procedure was undertaken with additional transverse firings of staplers between previously lengthened segments and tapering of redundant blind-ending portions of bowel.

Results

At operation, the bowel lengths were 45 and 62 cm. The second STEP left the patients with 61 and 73 cm of small bowel with a normalization of intestinal diameter. The first patient is doing well 5 months after surgery, and the second child tolerated increased enteral intake but died 1 year later from total parenteral nutrition-related liver failure.

Conclusions

This is the first report of a successful application of a second STEP procedure to further lengthen small bowel in SBS patients. Reapplication of the procedure requires careful stapling and a removal of small blind-ending segments to avoid further stasis.     

Serial transverse enteroplasty enhances intestinal function in a model of short bowel syndrome

OBJECTIVE/SUMMARY BACKGROUND DATA: Serial transverse enteroplasty (STEP) is a new intestinal lengthening procedure that has been shown to clinically increase bowel length. This study examined the impact of the STEP procedure upon intestinal function in a model of short bowel syndrome. METHODS: Young pigs (n=10) had a reversed segment of bowel interposed to induce bowel dilatation. Five pigs underwent a 90% bowel resection with a STEP procedure on the remaining dilated bowel while 5 served as controls and had a 90% bowel resection without a STEP procedure. Determinations of nutritional status, absorptive capacity, and bacterial overgrowth were conducted 6 weeks after resection. Statistical comparisons were made by 2-sample t test (significance at P<0.05). RESULTS: The STEP procedure lengthened the bowel from 105.2+/-7.7 cm to 152.2+/-8.3 cm (P<0.01). The STEP animals showed improved weight retention compared with controls (mean, -0.5%+/-1.8% body weight versus -17.6%+/-1.5%, P<0.001). Intestinal carbohydrate absorption, as measured by d-Xylose absorption and fat absorptive capacity as measured by serum vitamin D and triglyceride levels, were increased in the STEP group versus controls. Serum citrulline, a marker of intestinal mucosal mass, was significantly elevated in the STEP pigs compared with controls. None of the STEP animals but 4 of 5 control animals were noted to have gram-negative bacterial overgrowth in the proximal bowel. CONCLUSIONS: STEP improves weight retention, nutritional status, intestinal absorptive capacity, and serum citrulline levels in a porcine short bowel model. A salutary effect upon bacterial overgrowth was also noted. These data support the use of this operation in short bowel syndrome.     

Intermediate outcomes after serial transverse enteroplasty in children with short bowel syndrome

Background

Serial transverse enteroplasty (STEP) is a novel intestinal lengthening procedure introduced in 2003. To date, no human studies exist that report objective assessment of intestinal absorptive capacity. The aim of this study was to report intermediate outcomes in patients who have received the STEP at our institution using clinical and biochemical assessment of intestinal function.

Methods

All 14 patients who received the STEP since May 2003 were reviewed. Clinical (weight gain, enteral tolerance, stool frequency) and biochemical (citrulline levels, d-xylose absorption, alpha-1 antitrypsin clearance, and fecal fat content) outcomes were performed pre-STEP and post-STEP at 1, 6, and 12 months, respectively. Data are presented as means with standard deviation. Paired t tests were used to compare post-STEP outcomes with pre-STEP values (P < .05 was significant). Three patients had a STEP as a newborn and are analyzed separately.

Main Results

There were 14 patients (3 females; mean age, 24.8 months; range, 1 day-14 years). Serial transverse enteroplasty resulted in a mean increase in length of dilated bowel segment of 94% ± 30% and increase in total small bowel length of 49% ± 42% with mean application of 16 ± 9 cartridges and cost of Can$2878.51 ± 1406.22. Patients demonstrated improvement in both clinical parameters, as well as intestinal absorptive function. Complications included 2 patients with staple line leak and 1 patient with gastrointestinal bleeding from staple line ulcers. Three patients died (2 from liver failure and 1 from sepsis and congenital heart disease). Two patients received liver-intestinal transplants at 4 and 5 months post-STEP. Mean follow-up was 23 ± 9 months, with 7 patients followed more than 2 years. Of 8 patients with post-STEP follow-up of more than 1 year, 7 have weaned from parenteral nutrition.

Conclusion

Clinical and objective biochemical outcomes of intestinal function after the STEP procedure show promise after intermediate follow-up.     

Repeat surgical bowel lengthening with the STEP procedure

BACKGROUND: Feasibility of repeat lengthening has been demonstrated in animals, but in humans, none of the three clinical case reports have achieved enteral autonomy after secondary lengthening with serial transverse enteroplasty (STEP). PATIENTS AND METHODS: Institutional Review Board approved retrospective review of a single center series of repeat intestinal lengthening after prior Bianchi lengthening or STEP. Outcome measures examined include patient survival, weaning from parenteral nutrition, and need for intestinal transplantation. RESULTS: Repeat lengthening with STEP was performed 16 times in 14 patients including 2 adults and 7 male patients after prior Bianchi (n=7) and prior STEP (n=7). Median time after initial lengthening to reSTEP was 12 months (2 months-15 years). Median remnant bowel length before initial lengthening was 35 cm (15-110), before reSTEP was 56.5 cm (27-100) and final length after reSTEP was 90 cm (39-120). Survival is 100% with a median follow-up of 14.5 months (1-65 months). Discontinuation of parenteral nutrition (PN) after reSTEP was achieved in 6 of 14 (43%) patients at a median of 3 months (0.5-13 months) after reSTEP. Intestinal transplant salvage was performed in four patients at 7, 9, 13 months, and 4.6 years, respectively, after reSTEP. All four of those undergoing intestinal transplantation have also subsequently discontinued PN. CONCLUSIONS: Repeat lengthening is technically feasible after either of the available surgical lengthening procedures (Bianchi and STEP). In the stable patient, who is unable to wean from PN, repeat lengthening led to discontinuation of TPN in almost half of these carefully selected patients and does not preclude intestinal transplantation.     

Comparison of intestinal lengthening procedures for patients with short bowel syndrome

OBJECTIVE: Review the clinical results of 24 years of intestinal lengthening procedures at one institution. METHODS: Retrospective review of a single center experience comparing the outcome of 2 intestinal lengthening procedures (Bianchi and serial transverse enteroplasty [STEP]) in terms of survival, total parenteral nutrition (TPN) weaning, and complications. RESULTS: Sixty-four patients, including 14 adults, underwent 43 Bianchi and 34 STEP procedures between 1982 and 2007. Three patients had prior isolated liver transplants. The median (range) remnant bowel length before first lengthening was 45 (11-150) cm overall; (Bianchi=44 cm, STEP=45 cm) and 68 (20-250) cm after lengthening; (Bianchi=68 cm, STEP=65 cm). Actual survival is 91% overall (Bianchi 88%, STEP 95%) with median follow-up of 3.8 years (Bianchi=5.9 years, STEP=1.7 years). Average enteral caloric intake in pediatric patients was 15 kcal/kg before lengthening and 85 kcal/kg at 1 year after lengthening. Sixty-nine percent of patients are off TPN at most recent follow-up, including 8 who were weaned from TPN after intestinal transplantation. Liver disease (when present) was reversed in 80%. Surgical complications occurred in 10%, more commonly requiring reoperation after Bianchi than STEP. Intestinal transplantation salvage was required in 14% at a median of 2.9 years (range=8 months to 20.7 years) after lengthening. CONCLUSIONS: Surgical lengthening with both Bianchi and STEP procedures results in improvement in enteral nutrition, reverses complications of TPN and avoids intestinal transplantation in the majority with few surgical complications. Intestinal transplantation can salvage most patients who later develop life-threatening complications or fail to wean TPN.     

Five-year outcomes after serial transverse enteroplasty in children with short bowel syndrome

AimThe aim of this study is to report 5-year outcomes in patients who have received serial transverse enteroplasty (STEP) using clinical and biochemical assessments of intestinal function.MethodsThirty-one STEP procedures have been performed at our institution. Twelve patients who received STEP before January 2005 were analyzed. Clinical and biochemical outcomes were performed pre-STEP and post-STEP on an annual basis. Paired t tests were used to compare post-STEP outcomes to pre-STEP values (P < .05 was significant).Main ResultsThere were 12 patients (3 females; median age, 5.5 months). Serial transverse enteroplasty resulted in mean increase in length of dilated bowel segment of 89% ± 26% and increase in total small bowel length of 46% ± 40%. Two patients received liver-intestinal transplants post-STEP (at 4 and 5 months), and 2 patients died of liver failure at 3 and 8 months. The remaining 8 of 12 patients all show stable intestinal absorptive capacity (data not shown). Of 8 patients, 7 were weaned off parenteral nutrition by 4 years. No patient has required repeat STEP or bowel tapering. Two patients developed staple line leak, and 1 patient developed gastrointestinal bleed from staple line ulcers.ConclusionThis report represents the largest series of STEP patients followed up beyond 5 years. Long-term clinical, biochemical, and radiologic outcomes appear to be robust.     

The First Clinical Application of the Spiral Intestinal Lengthening and Tailoring (Silt) in Extreme Short Bowel Syndrome

Aim

Spiral Intestinal Lengthening and Tailoring (SILT) invented by our team is a new technique that offers minimal mesenteric handling and a more physiological result compared to the STEP procedure. Its feasibility has been tested in animal models and now we report the first successful human application in extreme short bowel syndrome.

Materials and Methods

A 3-year-old girl suffered subtotal loss of her small bowel and ileocaecal junction as a result of midgut volvulus. Only 15 cm of jejunum remained intact. Parenteral nutrition (PN), gastrostomy feeding, controlled bowel expansion and SILT procedure were applied.

Results

The length of the jejunum increased from the initial 15 to 22 cm during 12 months of PN and bowel expansion. Eleven centimeter of distended bowel was further lengthened up to 20 cm by SILT giving a total small bowel length of 31 cm. Oral and gastrostomy feedings were commenced 5 days postoperatively. There were no surgical complications 6 months after the procedure. The patient’s liver function was preserved, she was weaned off PN, discharged from hospital, but remained on gastrostomy top up feeding. The net weight gain of the patient was 1,800 g 6 months after the procedure.

Conclusion

SILT procedure is a safe and feasible technique for human intestinal lengthening and tailoring.     

First report of the international serial transverse enteroplasty data registry: indications, efficacy, and complications

BACKGROUND: Serial transverse enteroplasty (STEP) is a novel surgical therapy for short bowel syndrome and is being used with increasing frequency worldwide. Because no single center is likely to obtain sufficient experience for meaningful analysis, we created the International STEP Data Registry to allow for larger, multicenter patient accrual and followup. This report describes patient characteristics, operative parameters, and early results of STEP in the first 38 patients enrolled in the International STEP Data Registry. STUDY DESIGN: After IRB approval, data were entered online through password-protected enrollment and followup forms. Patient and procedural characteristics were analyzed. Pre- and postoperative small bowel length and enteral feeding tolerance were compared with the paired t-test. RESULTS: Between September 1, 2004, and April 30, 2006, 19 centers from 3 countries enrolled 38 patients. Median followup from STEP procedure to analysis was 12.6 months (range 0 to 66.9 months). Indications for STEP were short bowel syndrome (SBS, n=29), bacterial overgrowth (n=6), and neonatal atresia (n=3). Mean small intestine length was substantially increased in all groups (68+/-44 cm versus 115+/-87 cm, p < 0.0001, n=27). Notable complications included intraoperative staple line leak (n=2), bowel obstruction (n=2), and fluid collection or abscess (n=3). Late outcomes included progression to transplantation (n=3) and mortality (n=3). For the short bowel syndrome cohort, enteral tolerance was notably increased from 31%+/-31% to 67%+/-37% of calories (p < 0.01, n=21). CONCLUSIONS: STEP has been performed at multiple centers with minimal complications and encouraging outcomes. Indications for the procedure have broadened beyond short bowel syndrome to include bacterial overgrowth and neonatal intestinal obstruction with dilated proximal intestine. Continued accrual and followup of patients in the International STEP Data Registry will elucidate the longterm safety and efficacy of the procedure, with the goal of improved patient selection and operative timing.     

Gastrointestinal bleeding as a complication of serial transverse enteroplasty

Purpose

Serial transverse enteroplasty (STEP) lengthens and tapers bowel in patients with intestinal failure. Evaluation and treatment of serious late gastrointestinal bleeding (GIB) in three STEP patients are described.

Methods

Patients participating in an interdisciplinary intestinal rehabilitation program were reviewed to identify those who underwent STEP and had GIB requiring transfusion.

Results

Of 296 patients, 23 underwent STEP, and 3 (13%) had subsequent GIB requiring transfusion. Diagnoses were vanishing gastroschisis/atresia, malrotation/atresia, and gastroschisis.. STEP was performed at ages 3–5 months, using 5–15 stapler-firings with an increase in mean bowel length from 39 to 62 cm. GIB was diagnosed 5–30 months post-op and resulted in 1–7 transfusions per patient. Endoscopy demonstrated staple-line ulceration in two patients and eosinophilic enterocolitis in the third. All were treated with enteral antibiotics, sulfasalazine, and luminal steroids. Those with ulcers responded to bowel rest, and the patient with eosinophilic enterocolitis stabilized with luminal steroids. In all three, hemoglobin levels improved despite persistent occult bleeding.

Conclusions

Significant GIB is a potential late complication of STEP. Endoscopy identified the underlying source of GIB in all three patients. A combination of enteral antibiotics, anti-inflammatory medications, and bowel rest was effective in treating post-STEP GIB, without the need for additional bowel resection.     

Spiral intestinal lengthening and tailoring — first in vivo study

Introduction

Spiral Intestinal Lengthening and Tailoring (SILT) offers a new opportunity for the surgical treatment of short bowel syndrome. SILT requires less manipulation on the mesentery than the Bianchi procedure and does not alter the orientation of the muscle fibers like serial transverse enteroplasty (STEP). This study reports the first SILT results in a surviving animal model.

Material and Methods

Vietnamese minipigs (n = 6) underwent interposition of a reversed intestinal segment to produce proximal small bowel dilation. Five weeks later the reversed segment was resected, and the wall of the dilated intestine was cut spirally at 45°–60° to its longitudinal axis. The bowel was lengthened longitudinally, and the spiral shaped intestinal wound was sutured. Five weeks later, the animals were explored, and the lengthened segments were measured. Haematoxylin and eosin, picrosirius, neuron specific enolase, S-100, C-kit, and immunohistochemistry were performed.

Results

Mean lengthening was 74.8% ± 29.5% and mean tailoring (lumen reduction) was 56.25% ± 18.8%. No instances of necrosis, perforation, suture break down, or peritonitis were observed in 6/6 animals. Four of six animals recovered uneventfully with viable lengthened segments. Statistical analysis showed no significant difference in length (p = 0,078) and width (p = 0,182) after 5 weeks. Two animals developed bowel obstruction due to narrowed lumen, adhesion, and strangulation after 14 and 24 days of surgery. In both animals the lumen was tailored by more than 70% to less than 1.5 cm diameter. The mucosa and the muscle layers in the operated segment had become hypertrophic, but the orientation of the circular and longitudinal muscle fibres remained normal after the SILT procedure. There were no signs of chronic ischemia or collagen accumulation after the SILT. The myenteric and submucosal plexuses and the Cajal cell network appeared normal.

Conclusion

The bowel remained viable macroscopically and microscopically after SILT, such that SILT may be an alternative or an addition to the present technical repertoire of intestinal lengthening. However the limitations of tailoring should be kept in mind.     

Intestinal lengthening and growth hormone in extreme short bowel syndrome: a case report.

Recombinant growth hormone (GH) has been used successfully to promote mucosal intestinal adaptation in adults with short bowel syndrome. Here the authors report the case of an infant with extreme short bowel syndrome successfully treated with intestinal lengthening and GH. The patient was born with intestinal atresia leaving only 12 cm of jejunum and 2 cm of ileum with ileocecal valve. A Bianchi procedure extended the length to 25 cm, but 6 months later she still received 50% of her nutritional requirements by parenteral route. A 4-week course of 0.5 U/kg of GH dramatically changed the situation allowing weaning of total parenteral nutrition (TPN) 2 weeks after the end of the treatment with striking nutritional improvement, decrease in the number of stools from 7 to 2 per day, and no secondary effects. The authors believe that GH deserves clinical trial in cases of extreme short bowel.     

Intestinal Bowel Lengthening in Children with Short Bowel Syndrome: Systematic Review of the Bianchi and STEP Procedures

Background:

It has been thirty years since Bianchi introduced the technique that made intestinal lengthening possible. The last three decades have seen lengthening procedures established as vital components of intestinal rehabilitation programs. The goal of the present study was to use a systematic literature review to determine patient outcomes for the two most commonly used lengthening procedures, the Bianchi procedure and the serial transverse enteroplasty procedure (STEP).

Methods:

Pubmed and Embase were searched using the terms “intestinal lengthening” and “bowel lengthening.” Patient outcomes were extracted from each relevant journal article on the basis of a set proforma. The results were combined to create overall mean outcomes. Mean outcomes were also calculated separately for the Bianchi procedure and STEP. Significance was tested with the independent t-test.

Results:

Overall survival for the last thirty years is 83 %. However, survival for the last fifteen years has been 89 %, with no significant difference between the two procedures. The Bianchi procedure has a higher rate of weaning patients who were static on parenteral nutrition with conservative measures: 55 % versus 48 %. In addition, the Bianchi procedure was associated with a higher rate of patients receiving transplants: 10 % versus 6 %. The STEP has a higher rate of complication. Length of follow-up is significantly longer for the Bianchi procedure: 76 versus 22 months. The impact that this differential could have had on our results must be considered.

Conclusions:

Outcomes for intestinal lengthening procedures are very good, and increasingly so. However, further analysis is required in order to fully understand the relative strengths and weaknesses of each procedure.     

Preservation of intestinal motility after the serial transverse enteroplasty procedure in a large animal model of short bowel syndrome

Introduction

Serial transverse enteroplasty (STEP) has been shown to improve bowel function in short bowel syndrome. The effect of the STEP procedure on intestinal motility is not known, but some have hypothesized that it could disrupt bowel innervation and thus impair intestinal motility.

Methods

Growing Yorkshire pigs (n = 7) underwent 3 operations at 6-week intervals: (1) reversal of 50 cm of jejunum, (2) 90% bowel resection ± STEP to the proximal dilated bowel (4 STEP, 3 control), and (3) implantation of serosal strain gauges. At each operation, baseline and post-octreotide small intestinal motility was studied with continuously perfused manometry catheters using non-anticholinergic anesthesia. In addition, awake monitoring was performed using strain gauge analysis 1 week after the third operation. Characteristics of phase III of the migrating motor complex (MMC) were compared between and within groups using t test, χ², and analysis of variance, with significance set at P < .05.

Results

Manometry data from the third surgery revealed no differences between groups or compared with baseline within groups for the presence and characteristics of phase III of the MMC. Specifically, the mean amplitude and frequency of phase III after octreotide, and both the mean baseline and mean octreotide-stimulated motility indices were equivalent. The duration of phase III after octreotide stimulation was significantly increased in the STEP animals, suggesting a potential benefit of the STEP procedure. Strain gauge analysis, performed in awake animals, confirmed no differences between the groups for basal and octreotide-stimulated characteristics of phase III of the MMC.

Conclusions

These preliminary data suggest that the STEP procedure in a porcine model of short bowel syndrome does not interfere with baseline or hormonally stimulated motility within the small bowel. These findings further support the STEP procedure as a safe option for the surgical management of short bowel syndrome.     

Success of Serial Transverse Enteroplasty in an Adult With Extreme Short Bowel Syndrome: A Case Report

Since its introduction as an alternative intestinal lengthening technique, serial transverse enteroplasty has been increasingly used as the surgical treatment of choice for children with refractory short bowel syndrome, but there have been few reports about the adult patients. This report describes the case of a 71-year-old man with a short bowel after distal gastrectomy with Billroth II reconstruction for gastric cancer, followed by extensive intestinal resection. The serial transverse enteroplasty operation was performed and lengthened the small intestine from 49 to 67 cm. The patient tolerated the procedure well and weaned off total parenteral nutrition. Liver function also improved. This case shows that the serial transverse enteroplasty procedure increases intestinal length. This procedure should be considered a surgical option for adult patients with extreme short bowel syndrome.Key words: Adult, Extreme short bowel syndrome, Intestinal lengthening, Serial transverse enteroplastyShort bowel syndrome (SBS) is caused by the anatomic or functional loss of a large segment of small intestine and is characterized by the inability to maintain protein energy, fluid, electrolyte, or micronutrient balances when on a conventionally accepted normal diet.¹ Surgical management of SBS focuses on the principles of bowel conservation and restoration of intestinal continuity, which includes a variety of procedures designed to delay intestinal transit, taper dilated intestine, lengthen the bowel, or replace the lost bowel with an intestinal transplant.^2,3 However, conservation principles often give way to practical considerations at the time of the initial surgery, resulting in resection of dilated intestinal segments or ostomy creation. Serial transverse enteroplasty (STEP) is an alternative approach that offers the potential benefits of promptly establishing intestinal continuity and avoiding further loss of intestinal absorptive area.⁴The serial transverse enteroplasty procedure was first described in 2003 as an alternative intestinal lengthening technique for patients with refractory SBS.⁵ Similar to other bowel lengthening procedures, STEP lengthens the intestine and narrows bowel diameter with the intent of improving peristalsis and preventing small bowel bacterial overgrowth, malabsorption, and sepsis.⁶ In contrast, STEP has gained favorability as its novel approach is intuitive, relatively easy to perform, poses minimal threat to intestinal blood supply, and can be utilized for varying degrees of bowel dilatation.^4,6 Accepted indications of STEP include intestinal failure and bacterial overgrowth refractory to maximal medical management, as well as neonatal intestinal atresias or obstructions with limited small bowel length and a dilated proximal segment. However, most reports of STEP were in children with SBS; there were few reports in adult patients. Herein, we report the successful clinical application of this procedure in an adult patient who suffered from proximal 85% of small intestine removed, remained total parenteral nutrition (TPN)–dependent, and was diagnosed with parenteral nutrition–associated liver disease (PNALD).     

The second STEP: the feasibility of repeat serial transverse enteroplasty

Background

Serial transverse enteroplasty (STEP) lengthens and tapers dilated bowel. Redilation of the STEP segment occurs in some patients with intestinal failure. The feasibility of a repeat STEP procedure in a pig model is evaluated.

Methods

Six pigs underwent reversal of an intestinal segment distal to the ligament of Treitz. At 6-week intervals after reversal, each animal had 2 STEP procedures on the bowel proximal to the reversed segment. Necropsy was performed up to 6 weeks after repeat STEP.

Results

Bowel length increased by 11.3 ± 3.9 cm and bowel diameter decreased from a mean of 5.3 ± 0.8 to 1.8 ± 0.4 cm (P < .0001) after the first STEP. After repeat STEP, bowel length increased by 16.7 ± 13.3 cm (P < .01), and the bowel was tapered from a mean of 5.4 ± 0.9 to 2.2 ± 0.4 cm (P < .01). Five pigs did well after repeat STEP, and 1 pig had early necropsy for bowel obstruction. None had histologic evidence of bowel ischemia in the repeat STEP segment.

Conclusions

A second STEP operation is feasible in a pig model and may be considered to optimize bowel length and function in select patients with intestinal failure.     

Serial transverse enteroplasty is associated with successful short-term outcomes in infants with short bowel syndrome

Background

The serial transverse enteroplasty (STEP) has been shown to improve nutritional indices in an animal model of short bowel syndrome. The aim of this study was to review short-term surgical and nutritional outcomes in the first cohort of infants to undergo the STEP procedure at our institution.

Methods

All patients who underwent the STEP procedure during a 26-month period from February 2002 to March 2004 were reviewed. Paired t tests were used for comparisons between values pre-STEP and post-STEP (P < .05 deemed significant). Data are expressed as mean and range.

Results

The STEP was performed on 5 patients, including 1 newborn. The STEP was used as a primary lengthening operation in 4 patients. Intestinal length was significantly increased in all patients with 18 (10-26) stapler applications. There were no perioperative complications and no evidence of intestinal leak or obstruction on routine postoperative contrast study. Nutritional follow-up was available on 3 subjects at 17 (11-26) months post-STEP. Percentage of enteral nutrition was significantly increased in these subjects (P < .05). One subject was fully weaned from total parenteral nutrition 6 weeks after the STEP, and bilirubin in another patient with profound cholestasis who had been listed for liver-small bowel transplant normalized after the STEP. An additional patient, with established cirrhosis before operation, underwent successful liver-small bowel transplantation 8 months after intestinal lengthening.

Conclusion

The STEP procedure is a simple bowel-lengthening procedure with promising early surgical and nutritional outcomes. Further data from a multicenter registry are needed to demonstrate its long-term efficacy.     

Total intestinal aganglionosis: a new technique for prolonged survival

Total small bowel aganglionosis is uniformly fatal; and prolonged nutritional treatment for the resulting severe short bowel syndrome in the absence of a therapy designed to achieve a functional bowel length has not been warranted. We report an operative technique, long segment small bowel myectomymyotomy, which has produced a functioning length of intestine capable of supporting ever increasing amounts of enteral nutrition. A term female was noted to have neonatal intestinal obstruction, and two laparotomies proved total colonic and near-total small bowel aganglionosis. At 2 months of age reoperation was done and the aganglionosis was proved to extend to 7 cm below the ligament of Treitz. From this transition zone to 10 cm distally, a myectomy was done removing a 1 cm wide length of seromuscular tissue to the level of the submucosa. From the distal end of the myectomy, another 40 cm of bowel received an antimesenteric border myotomy cutting to the submucosal level followed by spreading of the cut surface to a width of 1 cm. This left the patient with 55 to 60 cm of small bowel from the ligament of Treitz to the end of the myotomy at which point an end ostomy was created. The remainder of the small bowel was excised and the colon exteriorized as a mucous fistula. The patient was continued on total parenteral nutrition alone for ten days at which time small volume enteral feeds were introduced. By 5 months of age, 25% of calories were enteral; by 6 months, 33% of calories were enteral; and by 8 months, 45% of intake was enteral.(ABSTRACT TRUNCATED AT 250 WORDS)     

The evaluation of the nutritional condition and the absorptive capacity in long-term follow-up of infants with short bowel syndrome

The nutritional conditions and absorptive capacity of nine infants with short bowel syndrome were investigated during a long follow-up period from one and half years to 14 years and 7 months. The length of the residual small intestine ranged between 13 and 90cm. The nine infants had normal meals at home and did not suffer from persistent diarrhea. Most of the infants were thin. Nutritional conditions, for example, serum protein, triglycerides, vitamins, trace elements and plasma amino acids, were kept relatively well, and there were no symptoms of deficiency. The results of D-xylose absorption test had gradually improved but the absorptive capacity of sugars and amino acids observed by potential differences were within the normal limits or slightly subnormal except one infant. This indicated that the absorptive capacity of sugars and amino acids per a given area of the residual small intestine did not increase. From our examination the critical length of intestine might be in the neighborhood of 50cm. The infants left with less than 50cm of small intestine seem to have grown up normally but malabsorption of fat, sugars and bile acids have continued for many years.     

短肠综合征患者血清瓜氨酸水平与小肠吸收面积和功能的相关性

目的研究短肠综合征患者血清瓜氨酸水平的变化及其与肠道面积及吸收功能的相关性。方法采用高效液相色谱法测定22例短肠患者（短肠组）和33例健康人（对照组）血清瓜氨酸水平。短肠患者残存小肠长度及直径采用X线造影检测，并测定短肠患者尿D-木糖排泄率和肠道蛋白吸收度。分析短肠患者血清瓜氨酸与残存小肠长度、面积、蛋白及D-木糖吸收的相关性。6例行肠康复治疗的患者测定康复治疗前后瓜氨酸、D-木糖及蛋白吸收水平的变化。结果短肠组血清瓜氨酸水平显著低于健康对照组[（5．94±2．65）比（16．87±5．97）μmol／L，P〈0．01]。短肠组患者血清瓜氨酸水平与残存小肠长度（r=0.82）及表面积（r=0.86）呈正相关，与尿D-木糖排泄（r=0.56）及肠道蛋白吸收（r=0.48）也呈正相关。6例行肠康复治疗的患者治疗后血清瓜氨酸水平、蛋白及D-木糖吸收均显著增加，但3者增加百分比之间并无相关。结论血清瓜氨酸水平与短肠患者的小肠吸收面积和吸收功能呈正相关，能反映短肠患者小肠功能和衰竭程度，是康复疗效的良好指标。