Can levels of interleukins and matrix metalloproteinases in the amniotic fluid predict postnatal bowel function in fetuses with gastroschisis?

Purpose

The aim of this study was to determine whether interleukin or matrix metalloproteinase (MMP) levels in the amniotic fluid can predict the postnatal condition of the bowel in fetuses with gastroschisis (GS).

Methods

Eleven pregnant women with a fetus with GS underwent amniocentesis. Levels of IL-1α, IL-1β, and IL-6 were measured in 10 of 11 specimens, and MMP-1, MMP-3, and MMP-8 levels were measured in 7 of 11 specimens. Neonates who tolerated complete oral feeding within 21 days were considered to have good bowel status (GS/good: n = 4, 4 in interleukin study or 3 in MMP study); those who did not were considered to have poor bowel status (GS/poor: n =7, 6 in interleukin study or 4 in MMP study). Seventy-eight women undergoing amniocentesis for other reasons served as controls.

Results

Interleukin-1 levels were considerably lower in the pregnancies complicated by GS than in controls. Matrix metalloproteinases 3 and 8 levels were significantly higher in the GS pregnancies than in controls. Neonates with poor bowel status had higher MMP-3 and MMP-8 levels than those with good bowel status.

Conclusion

Amniotic fluid MMP-3 and MMP-8 levels may prove useful to assess the condition of the bowel in fetuses with GS.     

Gastroschisis, atresia, dysmotility: surgical treatment strategies for a distinct clinical entity

Purpose

Intestinal atresia (IA) occurs in 10% to 20% of infants born with gastroschisis (GS). We describe a distinct subset of these children with severe dysmotility, without mechanical intestinal obstruction, and with adequate intestinal length for enteral nutrition and propose possible treatment strategies.

Methods

A total of 177 patients with GS managed at a single institution between 1993 and 2007 were retrospectively reviewed.

Results

Twenty-one (12%) patients had IA. Six (29%) did well, with gradual progression to full diet; 1 died at birth because of complete midgut infarction; 1 died of necrotizing enterocolitis; 4 with short bowel syndrome (SBS) (small bowel length, 30-41 cm) died of liver failure because of total parenteral nutrition (3) or sepsis (1). Nine (43%) were felt to have gastroschisis/atresia/dysmotility, defined as adequate small bowel length for survival as measured at time of laparotomy (mean, 146 cm; range, 66-233 cm), massive intestinal dilatation, and stasis. Of 9 patients with gastroschisis/atresia/dysmotility, 5 (56%) survived. All had surgery to “rescue” their dysfunction intestine, at mean age 128 days (range, 52-271 days): 4 had tapering enteroplasties of mean 37 cm (range, 5-115 cm)—all 4 survived; 3 had diverting stomas created for intestinal decompression, followed by stoma closure—1 survived; 1 nonsurvivor had a Kimura “patch”; 1 nonsurvivor underwent redo anastomosis. Survivors weaned off total parenteral nutrition at mean age 331 days after rescue surgery (range, 42-814 days).

Conclusions

More than one third of patients with GS/IA appear to have significant intestinal dysmotility without true SBS nor obstruction. Successful treatment of these infants may be achieved with the use of tapering enteroplasty and/or temporary diverting stomas.     

Intestinal transplantation for short bowel syndrome secondary to gastroschisis

Background/Purpose

Gastroschisis is the most frequent cause of pediatric intestinal transplantation. This study reviews our experience of intestinal transplantation secondary to gastroschisis to elucidate those factors affecting the outcome of children with short bowel syndrome.

Methods

A retrospective review was performed for children who underwent intestinal transplantation for gastroschisis at the University of Miami between June 2003 and August 1994.

Results

Thirty-two transplants were performed in 28 children with gastroschisis during the study period. Associated intestinal anomalies were present in 22 infants (atresia [n = 14], volvulus [n = 3], and/or ischemia [n = 16]). Spontaneous prenatal closure of gastroschisis, a rare anomaly associated with bowel atresia and ischemia because of a very small abdominal defect, was seen in 9 patients. Most of the patients had a complicated course and required multiple abdominal surgeries before transplant. Fifteen (53.6%) patients are currently alive at a median follow-up of 23.5 months. Short-term survival rate has significantly improved in recent years.

Conclusions

Patients with complex gastroschisis and intestinal anomalies have a significant risk for progression to short bowel syndrome. Intestinal transplantation can be a lifesaving option and provides a satisfactory outcome for children with short bowel syndrome secondary to gastroschisis.     

Gastrointestinal ramifications of the cloacal exstrophy complex: a 44-year experience

Purpose

Cloacal exstrophy is a rare and complex congenital anomaly requiring coordination among multiple pediatric subspecialties. There is currently no consensus regarding the fate and function of the hindgut, which plays an integral role in patients' long-term gastrointestinal health and genitourinary reconstruction.

Methods

A retrospective chart review was performed evaluating 77 patients with cloacal exstrophy treated during the previous 44 years at our institution.

Results

Seventy-seven patients with cloacal exstrophy were treated between 1965 and 2008. Sixty-five were white, 6 were African American, 3 were Asian, and 3 were Hispanic. Genotypes included 44 XY, 32 XX, and 1 XYY. Fifty-one were reared as females and 26 as males. The hindgut length was 2 to 5 cm in 11 patients, 6 to 10 cm in 18 patients, 11 to 15 cm in 6 patients, 16 to 20 cm in 7 patients, and greater than 20 cm in 2 patients. The hindgut length was unknown in 33 patients. Forty-seven patients had tubularization of the cecal plate with an end colostomy, and 30 patients had an ileostomy placed for bowel diversion purposes. Four patients had short gut syndrome. Thirty-one patients had genitourinary reconstruction, 12 using small bowel and 19 using colon. Eight patients had hindgut pull-through procedures.

Conclusion

Gastrointestinal ramifications of the cloacal exstrophy complex include the occurrence of short gut syndrome and significant fluid and electrolyte derangements in patients receiving an ileostomy for initial intestinal management. This has caused a paradigm shift of initial intestinal management to tubularization of the cecal plate with end colostomy placement. This shift has eliminated the occurrence of short gut syndrome and enabled patients to be candidates for intestinal pull-through procedure if these patients are able to form solid stool, have a reasonable degree of pelvic neuromuscular development, and are able to comply with a bowel management program.     

Closed gastroschisis: total parenteral nutrition-free survival with aggressive attempts at bowel preservation and intestinal adaptation

Background

In infants with gastroschisis antenatal closure of the umbilical defect results in a proximal atresia with ischemia and/or volvulus of the extracorporeal midgut. It has been described as “closed gastroschisis” or “vanishing midgut.”

Methods

A 10-year review of 219 gastroschisis patients identified 10 infants with this rare complication.

Results

In these 10 infants, the extracorporeal midgut was invariably matted and fibrosed. In 3 cases, the midgut had completely “vanished.” In the remaining 7 cases, the remnant midgut was surgically reduced into the abdominal cavity with care not to compromise the diminutive vascular pedicle. Abdominal exploration was performed several weeks later to reestablish bowel continuity; 4 required an ostomy and 2 underwent a serial transverse enteroplasty. Mean residual length of salvaged small bowel was 79 cm with retention of the distal half of the colon. Eight infants survived the initial hospitalization, with a mean length of stay of 121 days and mean hospital charge of $287,094. Six of the 7 long-term survivors have been completely weaned off total parenteral nutrition.

Conclusion

A nihilistic attitude toward infants with closed gastroschisis may not be uniformly supported because in the majority of these infants' long-term independence from total parenteral nutrition was achieved.     

Antenatal diagnosis of bowel dilatation in gastroschisis is predictive of poor postnatal outcome

Purpose

Although gastroschisis infants usually have a good outcome, there remains a cohort of babies who fare poorly. We inquired whether the presence of bowel dilatation in utero is predictive of postnatal course in infants with gastroschisis.

Methods

We compared the clinical course of infants who had bowel dilatation with those who did not. Bowel dilatation was defined as more than 20 mm in cross-sectional diameter on ultrasound at any gestational age. Outcome measures used were length of time of parenteral nutrition, death, and surgery for intestinal failure.

Results

A review of 170 infants with gastroschisis identified 74 who had dilatation of more than 20 mm (43.5%). There was no significant difference in the incidence of intestinal atresia in those with bowel dilatation and those without (P = .07). Those with bowel dilatation spent a longer period on parenteral nutrition. There were significantly more deaths in the group with bowel dilatation (P = .01). There was no significant difference in the number of infants requiring surgery for intestinal failure between the 2 groups (P = .47).

Conclusions

We found that sonographically detected bowel dilatation more than 20 mm in utero in fetuses with gastroschisis may have value in predicting clinically significant adverse postnatal outcomes.     

Surgical experimental jejunoileal atresia in rat embryo

Purpose

Jejunoileal atresia represents about 40% of intestinal atresia. After surgical repair, intestinal motility disorders are burdened with the postoperative outcome, and the origin of these troubles remains unclear. To specify the physiopathologic feature of jejunoileal atresia, we developed an experimental surgical model in fetal rat.

Methods

Time-dated pregnant rats were operated on at 18 days of gestational age. Hysterotomy was performed, followed by fetal wall incision. The exteriorization of the bowel loop was obtained by saline injection; the intestine was ligated and returned to the abdominal cavity before incisions were closed. Fetal intestine was excised at day 21, after cesarean delivery.

Results

Twenty-one pregnant rats underwent operation with 90% maternal survival rate. Among the 56 fetuses successfully operated on, 49 survived (87%). In fetuses with atresia, the mean birth weight (4.5 ± 0.6 g) and the mean intestinal length (12.8 ± 1.3 cm) were significantly lower compared to sham fetuses and controls.

Conclusion

The rat model offers the advantage of a low-expense mammal model with a wide panel of probes and reagents available for the study of the gut. This model of jejunoileal atresia could be used to study the consequences of prenatal intestinal obstruction on fetal gut.     

A serum factor(s) after small bowel resection induces intestinal epithelial cell proliferation: effects of timing,site, and extent of resection

Background/purpose

After small bowel resection (SBR), serum induces proliferation in rat intestinal epithelial cells (RIEC-6). This study was designed to elucidate the effects of postoperative time interval, site, and magnitude of SBR on RIEC-6 proliferation.

Methods

Serum was collected from rats at various times after a 75% mid-SBR or sham operation and added to RIEC-6 cells and growth determined over 5 days. In other experiments, cell growth was recorded in the presence of serum from rats after 25%, 50%, or 75% SBR, or after jejunal or ileal SBR.

Results

SBR serum enhanced RIEC-6 cell proliferation as early as 12 hours after resection. The extent of SBR directly correlated with the level of adaptation; however, the effects on cell growth by the serum were similar. SBR serum induced proliferation equally after either proximal or distal resection.

Conclusions

Serum contains a factor that stimulates intestinal cell proliferation soon after SBR but independent of the degree or site of intestinal resection. Although humoral factor(s) play a role in the early induction of enterocyte proliferation after SBR, further modulation of adaptation to varied lengths or sites of intestinal resection are probably governed by mechanisms independent of factors that circulate in the serum.     

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.     

Definitive percutaneous treatment of lymphatic malformations of the trunk and extremities

Purpose

The aim of this study is to investigate a new treatment regimen for macrocystic and microcystic lymphatic malformations (LMs) of the trunk and extremities.

Methods

Sixteen patients (aged 2 months-22 years) underwent percutaneous treatment for LM of the trunk and extremities from 2002 to 2007. The LM involved the arm, leg, axilla, chest, abdomen, scrotum, and penis. Eleven patients underwent primary treatment of LM; 5 were treated for recurrence after prior operative resection. Macrocysts (≥1 cm) were treated with dual-drug chemoablation (sequential intracystic sodium tetradecyl sulfate and ethanol); doxycycline was used for microcysts. Macrocysts and microcysts were treated after complete cyst aspiration using sonographic guidance. Twenty-four macrocysts and 103 microcysts were treated. The goal of treatment was complete cyst ablation documented by sonography or magnetic resonance imaging.

Results

The mean number of treatments was 1.7 per patient; the mean number of treatments for macrocysts was 1.3 and for microcysts was 1.7. Ablation efficacy was 100% (127/127 cysts). Treatments included massive intraperitoneal cysts and cysts surrounding the adventitia of the brachial artery and brachial nerve. Infection occurred in 2 (13%) of 16 patients. No patient experienced postprocedural pain, skin necrosis, neuropathy, bowel obstruction, skin retraction, or myoglobinuria.

Conclusions

Percutaneous sclerotherapy provides effective treatment for macrocystic and microcystic LM as primary treatment or for recurrence after surgical resection.     

Etiology and one-year follow-up results of hearing loss identified by screening of newborn hearing in Japan

Objective

To evaluate the incidence of newborn hearing loss in a Japanese population and to elucidate etiological factors and one-year prognosis.

Study Design

Screening of newborn hearing.

Setting

Children's tertiary referral center.

Subjects and Methods

Between 1999 and 2008, 101,912 newborn infants were screened, with 693 infants (0.68%) referred. Etiology investigation included CT, detection of cytomegalovirus (CMV) DNA, and connexin 26 mutation.

Results

Abnormal results (auditory brainstem response [ABR] threshold ≥ 35 normal hearing level [dB nHL] in either side) were observed in 312 infants (0.31%), and 133 subjects (0.13%) with ABR thresholds ≥ 50 dB nHL on both sides were classified into the habilitation group. In this group, inner ear/internal auditory meatus anomalies were detected in 20 of 121 subjects (17%) tested, middle/external ear anomalies in 14 of 121 subjects (12%), CMV DNA in 13 of 77 subjects (17%), and connexin 26 mutation in 28 of 89 subjects (31%). In 68 subjects undergoing all three investigations (CT, CMV, and connexin 26), 41 (60%) had positive results in at least one test. With inclusion of otitis media with effusion and perinatal problems, this rate amounted to 78% (53 subjects). Of the 97 infants in the habilitation group successfully followed up to one year, 36 (37%) showed a threshold change of 20 dB or more in either ear: 11 (11%) progression and 25 (26%) improvement, and 15 infants (15%) were reclassified into a less severe classification.

Conclusion

Considering that 26 percent of infants with bilateral moderate to severe hearing loss showed improvement in one year, habilitation protocols, especially very early cochlear implantation within one year of birth, should be reconsidered.     

Outcomes of the “clip and drop” technique for multifocal necrotizing enterocolitis

Background

The “clip and drop” (CD) has been proposed as a useful strategy in the management of severe multifocal necrotizing enterocolitis (NEC). There is little published data on clinical outcomes after this intervention. We report a 2-center experience with this technique.

Methods

A retrospective review of infants who underwent CD between 1998 and 2006 at 2 tertiary pediatric surgery centers. Data recorded included intestinal resections, interval between laparotomies, anastomoses at subsequent surgery, time to full feeds, and complications including mortality. Data are reported as median with ranges.

Results

Thirteen infants (7 male, 6 female) with a birth weight of 811 (514-2110) g underwent CD of up to 5 bowel segments. In 8 of 9 early survivors, all CD segments were viable. Six patients (46%) were alive at 29 (9-96) months. Survivors underwent 4 (3-4) laparotomies and 4 (2-6) bowel anastomoses and had intestinal continuity restored at 67 (51-162) days.

Conclusions

With multiple interventions, half the infants in this high-risk group survived and achieved full enteral feeds. The CD is a valuable technique in a selected group of infants with fulminant NEC.     

Neonatal outcomes associated with intestinal abnormalities diagnosed by fetal ultrasound

Purpose

Intestinal abnormalities are sometimes seen during antenatal testing; however, the postnatal importance of these findings has not been well established. We evaluated whether abnormal intestinal appearance on fetal ultrasound (US) was ultimately related to neonatal outcome.

Methods

Fetal US examinations from 2003 to 2006 were evaluated. Hyperechogenic bowel was defined as having the echogenicity comparable to bone, and dilated bowel was identified based on the sonographer's assessment. Persistence or resolution of US findings on subsequent US examinations and eventual outcomes were assessed. Cases were categorized as hyperechogenic or dilated and then subgrouped based on whether the US finding resolved.

Results

Sixty-eight fetuses had either hyperechogenic (n = 48) or dilated bowel (n = 20) on antenatal US. In 56 cases, complete data were available for analysis. Of 44 liveborn infants, 11 (25.0%) had an abdominal abnormality, and 33 (75.0%) were normal at birth. Compared to those with dilated bowel, fetuses with hyperechogenic bowel had a higher rate of prenatal demise (20.8% vs 10%) but a lower rate of abnormality at birth (10.3% vs 53.3%). Hyperechogenic bowel resolved on subsequent US more frequently than dilated bowel (65.5% vs 20.0%). In both groups, all fetuses with sonographic resolution were normal at birth. Of 9 fetuses that had persistently hyperechogenic bowel, 3 (33.3%) were born with an abnormality, and all were found to have meconium peritonitis or meconium ileus. In the 12 cases where dilated bowel did not resolve, 8 (66.7%) were eventually born with an abnormality, most commonly intestinal atresia.

Conclusions

Hyperechogenic and dilated bowel are associated with a significant rate of fetal demise. Hyperechogenicity is more common than dilation and is more likely to be transient. Dilated bowel is more often associated with neonatal abnormality than hyperechogenic bowel. Persistence of fetal US findings predicts a higher likelihood of abnormality in the neonate.     

A population-based study of the outcome after small bowel atresia/stenosis in New South Wales and the Australian Capital Territory, Australia, 1992-2003

Purpose

The purpose of the study was to describe the incidence, epidemiology, and survival of infants with small bowel atresia/stenosis in New South Wales (NSW) and the Australian Capital Territory (ACT), Australia.

Methods

A population-based cohort study was conducted of infants diagnosed with small bowel atresia/stenosis in NSW and the ACT from 1992 to 2003. Data were obtained from the prospectively collated NSW and ACT Neonatal Intensive Care Units' data collection. Individual risk factors for mortality were assessed using the χ² test.

Results

The incidence of small bowel atresia/stenosis in NSW and the ACT was 2.9 per 10,000 births. Of 299 infants identified with small bowel atresia, 13 were stillborn. Of the 286 live born infants, most (52%) were delivered preterm (<37 weeks' gestation) with an 87% survival, whereas 48% were term with a 98% survival. More than half the infants (54%) had an associated birth defect. The overall mortality was 8%. Prematurity and low birth weight were identified as independent risk factors for mortality (P < .001).

Conclusions

This study of small bowel atresia/stenosis provides population-based outcomes for clinicians and families. It is important to investigate infants with small bowel atresia for associated birth defects. Although the mortality rate has decreased over the last 50 years, it remains substantial at 8% and is higher in premature and low birth weight infants.     

A report of 6 children with small bowel intussusception that required surgical intervention

Background/Purpose

We aim to justify the need for surgical intervention in our patients with childhood small bowel intussusceptions (SBIs) and review the current concepts in childhood SBI.

Materials and Methods

We retrospectively reviewed the clinical charts of all patients with surgically confirmed SBI between July 1999 and October 2002. Demographic data, clinical presentation and investigations, operative and pathologic findings, and outcome were analyzed.

Results

Of 173 patients with intussusception, 6 (3.5%) were diagnosed with SBI. Median age was 11 months. Ultrasonography revealed intussusceptions in all patients, but only 1 was diagnosed with SBI. Air enema reductions were attempted in 4 of 6 patients with all ending up in failure and surgery. Surgery revealed ileoileal intussusceptions in 4 patients and jejunojejunal intussusceptions in 2 patients. Two patients had long intussusceptions measuring between 30 and 50 cm in length. Five patients had pathologic lead points, and bowel complications occurred in 2 patients. All underwent bowel resection and primary anastomosis.

Conclusion

Despite reports on spontaneous reduction of SBI, surgery was unavoidable in all our patients with SBI because of the presence of pathologic lead points and/or bowel complications. Air enema reduction was ineffective in SBI. Due caution should be exercised when selecting patients for expectant management.     

Effects of Cyclosporine on the Antioxidant Status and Oxidative Stress in the Glioma Cells

Objectives

After organ transplantation, some patients suffer neurological complications. Among the immunosuppressants, cyclosporine can cause neurological side effects. However, the mechanisms of encephalopathy by cyclosporine are not fully understood. We measured the antioxidant status, the hydrogen peroxide level, and the malondialdehyde level in glioma cells after cyclosporine treatment.

Methods

The production of hydrogen peroxide was determined using a modified xylenol orange method. The amount of malondialdehyde was measured using the thiobarbituric acid assay. Total antioxidant status was measured using Free Radical Analytical System 4 with kits.

Results

Cyclosporine resulted in the production of hydrogen peroxide by the glioma cells. The increased production of hydrogen peroxide depended on the drug concentration. The antioxidant status was decreased in the glioma cells after cyclosporine treatment. The malondialdehyde level was not changed in glioma cells after cyclosporine treatment.

Conclusions

Increased production of reactive oxygen species and decreased antioxdant status by cyclosporine in glioma cells may contribute to neurological side effects in transplantation patients.     

Effect of sex and sex hormones on structural intestinal adaptation after massive small bowel resection in rats

Purpose

The gonadal steroids play a major role in the regulation of many functions. The purpose of the current study was to evaluate the effect of sex and sex hormones on intestinal adaptation in a rat model of short bowel syndrome (SBS).

Methods

In the first experiment, male and female Sprague-Dawley rats underwent bowel transection and re-anastomosis (sham group) or 75% small bowel resection and anastomosis (SBS group). Relative changes in parameters of intestinal adaptation (overall bowel and mucosal weight, mucosal DNA and protein, villus height, and crypt depth) were measured on day 15 and were compared with respect to sex. In the second experiment, male rats were divided into 4 experimental groups: SBS rats, SBS castrated rats, SBS castrated rats treated with testosterone, and SBS castrated rats treated with estradiol. Parameters of intestinal adaptation were compared with respect to hormonal treatment. Statistical significance was determined by Student's t test and analysis of variance with P < .05 considered significant.

Results

Sex had minimal effects on intestinal adaptation. Both male and female rats showed a comparable increase in all parameters of intestinal adaptation. In the second experiment, castration led to significant decrease in bowel and mucosal weight, mucosal DNA and protein in both jejunum and ileum compared with SBS animals. Castrated rats also had lower jejunal villus height and crypt depth compared with SBS animals. Testosterone attenuated this negative effect of castration on bowel regrowth. Rats treated with testosterone showed a significant increase in bowel and mucosal weight, mucosal protein in both jejunum and ileum, mucosal DNA, villus height, and crypt depth in jejunum compared with castrated nontreated animals. Treatment with estradiol after resection and castration had minimal effect on bowel regrowth.

Conclusions

Bowel regrowth after massive small bowel resection is not sex-related. Depletion of androgens by castration inhibited intestinal adaptation. Testosterone has shown a strong stimulating effect on bowel regrowth.     

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.