Long-Term Outcomes of Short Bowel Syndrome Requiring Long-Term/Home Intravenous Nutrition Compared in Children With Gastroschisis and Those With Volvulus

doi:10.1016/j.transproceed.2009.12.033

Transplantation Proceedings

Volume 42, Issue 1, January–February 2010, Pages 5-8

https://doi.org/10.1016/j.transproceed.2009.12.033 Get rights and content

Abstract

Introduction

Short bowel syndrome (SBS) is a malabsorptive state that occurs following extensive small intestinal resection. The most severely affected children require intravenous feeding/parenteral nutrition (PN). Two common causes in infancy/early childhood are intestinal resection for volvulus and resection of necrotic small intestine in infants with gastroschisis. The aim of this study was to review the long-term outcomes of children with severe intestinal failure who remained dependent on PN for many months or years. We evaluated the outcomes among gastroschisis cases versus those following resection of mid-gut volvulus or congenital short gut.

Methods

We reviewed the case records over a 10-year period from 1997 of children presenting by 5 years of age with volvulus and/or congenital short gut or gastroschisis and who were dependent on long-term/home PN. We obtained data regarding the diagnosis, the small intestinal length, the presence of the ileo-cecal valve, the survivals, and the incidences of weaning from PN.

Results

Six children underwent resection following volvulus or congenital short gut syndromes and 7 had gastroschisis. Ten of the 13 children underwent surgery as neonates and 3 from 2–5 years of age. The residual small intestinal length was <10 cm in 1, 10–25 cm in 4, 25–50 cm in 5, 50–100 cm in 2, and >100 cm in 1 case. The ileo-cecal valve was removed in 8 children. All children were treated with PN after surgery for at least 7 months. All 7 children with gastroschisis and 1 with <10 cm small intestine after intestinal resection for volvulus still required PN after 14–120 months. Three with gastroschisis died. All 5 children with volvulus and >10 cm small intestine were weaned from PN, thriving at our review 7 months–7 years later.

Conclusions

Infants and young children with short gut and >10 cm small intestine gain intestinal autonomy. In contrast, those with gastroschisis have poor outcomes and should be referred for intestinal transplantation assessment more readily than those with after intestinal resection for volvulus short gut.

Section snippets

Patients and Methods

We examined the case records of all children discharged home on intravenous nutrition with short gut due to gastroschisis or volvulus aged 5 years and younger at the time of surgery. We reviewed the records of subjects presenting over a 10-year period from 1997 to 2007. Data were collected regarding the surgical procedure, the residual small bowel length, and the presence/absence of an ileo-cecal valve. Outcomes were assessed with regard to long-term intestinal failure as well as ability to be

Results

There were 13 children discharged home on PN treatment from 1997–2007. Nine (64%) were boys and 4 were girls. Patient details and outcomes are shown in Table 1.

Ten children with intestinal failure were on treatment with intravenous nutrition from the neonatal period. Two underwent intestinal resection, commencing intravenous nutrition treatment when aged 2 years, and 1 when 5 years old. SBS was secondary to extensive surgical resection for volvulus in 5 (38%), congenital short gut in 2 (15%),

Discussion

Children with gastroschisis who needed long-term/home intravenous feeding for months or years displayed worse outcomes than those aged 5 years and under at presentation with congenital or after intestinal resection for volvulus short gut. All infants and children with after intestinal resection for volvulus short gut and >10 cm small intestine successfully gained intestinal autonomy. The only deaths were in the group with gastroschisis.

The outcomes for infants and children with severe

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Parenteral support and micronutrient deficiencies in children with short bowel syndrome: A comprehensive retrospective study
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Short bowel syndrome (SBS) is the leading cause of chronic intestinal failure. The duration of parenteral support (PS) and the long-term micronutrient needs in children with SBS vary, based on their clinical and anatomical characteristics. Our study aimed to review the clinical course and identify high risk patient groups for prolonged PS and long-term micronutrient supplementation.
A retrospective review was conducted on electronic medical records of children with SBS and chronic intestinal failure who were enrolled in the multidisciplinary intestinal rehabilitation program at Manchester Children's Hospital, UK. Children were included in the review if they required PN for more than 60 days out of 74 consecutive days and had at least 3 years of follow-up. Statistical analysis was performed using IBM SPSS Statistics 24.0.
40 children with SBS achieved enteral autonomy (EA) and 14 remained dependent on PS after 36 months of follow up. Necrotizing enterocolitis was the most common cause for intestinal resection (38.9%) followed by gastroschisis (22.2%), malrotation with volvulus (20.4%), segmental volvulus (9.3%) and long segment Hirschsprung disease (1.9%). Those who achieved EA had significantly longer intestinal length 27.5% (15.0–39.3) than those who remained on PS 6.0% (1.5–12.5) (p < 0.001). Type I SBS was only found in the PS cohort. Median PN dependence was 10.82 months [IQR 5.73–20.78]. Congenital diagnosis was associated with longer PN dependence (21.0 ± 20.0) than acquired (8.7 ± 7.8 months), (p = 0.02). The need for micronutrient supplementation was assessed after the transition to EA; 87.5% children had at least one micronutrient depletion, most commonly Vitamin D (64.1%), followed by iron (48.7%), Vitamin B12 (34.2%), and vitamin E (28.6%). Iron deficiency and vitamin A depletion were correlated with longer PS after multivariate analysis (OR: 1.103, 1.006–1.210, p = 0.037 and OR: 1.048, 0.998–1.102, p = 0.062 respectively).
In our cohort, small bowel length was the main predictor for EA. Children on longer PS, had more often a congenital cause of resection and were at risk for micronutrient deficiencies in EA.
Long-term treatment with teduglutide: a 48-week open-label single-center clinical trial in children with short bowel syndrome
2023, American Journal of Clinical Nutrition
Short bowel syndrome (SBS) is the main cause of intestinal failure in children.
This single-center study evaluated the safety and efficacy of teduglutide in pediatric patients with SBS–associated intestinal failure (SBS-IF).
Children with SBS followed at our center with ≥2 y on parenteral nutrition (PN) and with small bowel length <80 cm who had reached a plateau were consecutively included in the study. At baseline, participants underwent a clinical assessment including a 3-d stool balance analysis, which was repeated at the end of the study. Teduglutide was administered subcutaneously 0.05 mg/kg/d for 48 wk. PN dependence was expressed as the PN dependency index (PNDI), which is the ratio PN non-protein energy intake/REE. Safety endpoints included treatment-emergent adverse events and growth parameters.
Median age at inclusion was 9.4 y (range: 5–16). The median residual SB length was 26 cm (IQR: 12–40). At baseline, the median PNDI was 94% (IQR: 74–119), (median PN intake: 38.9 calories/kg/d, IQR: 26.1–48.6). At week 24, 24 (96%) children experienced a reduction of >20% of PN requirements with a median PNDI = 50% (IQR: 38–81), (PN intake: 23.5 calories/kg/d IQR: 14.6–26.2), P < 0.01. At week 48, 8 children (32%) were weaned completely off PN. Plasma citrulline increased from 14 μmol/L (IQR: 8–21) at baseline to 29 μmol/L (IQR: 17–54) at week 48 (P < 0.001). Weight, height, and BMI z-scores remained stable. The median total energy absorption rate increased from 59% (IQR: 46–76) at baseline to 73% (IQR: 58–81) at week 48 (P = 0.0222). Fasting and postprandial endogenous GLP-2 concentrations increased at weeks 24 and 48 compared with baseline. Mild abdominal pain at the early phase of treatment, stoma changes, and redness at the injection site were commonly reported.
Increased intestinal absorption and PN dependency reduction were observed with teduglutide treatment in children with SBS-IF.
ClinicalTrials.gov NCT03562130. https://clinicaltrials.gov/ct2/show/NCT03562130?term=NCT03562130&draw=2&rank=1
Factors influencing enteral autonomy after autologous gastrointestinal reconstructive surgery: A two-centre UK perspective
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The implementation of multidisciplinary care and improvements in parenteral nutrition (PN) in patients with short bowel syndrome (SBS) have led to better outcomes and higher survivability. Autologous gastrointestinal reconstructive (AGIR) surgery can reduce the duration on PN and lead to earlier enteral autonomy (EA). Our aim was to investigate the effect of SBS aetiology and other predictors on the achievement of enteral autonomy following AGIR surgery.
Retrospective review of all patients undergoing AGIR surgery in two tertiary paediatric surgical units, between 2010 and 2021. Continuous data is presented as median (range).
Twenty-seven patients underwent 29 AGIR procedures (20 serial transverse enteroplasties (STEP), 9 longitudinal intestinal lengthening and tailoring (LILT)) at an age of 6.6 months (1.5 – 104.5). EA rate was 44% at 13.6 months after surgery (1 – 32.8). AGIR procedures achieved an increase in small bowel length of 70% (pre-operative 46.5 vs 77 cm, p = 0.003). No difference was found between STEP and LILT (p = 0.84). Percentage of expected small bowel length (based on the child's weight) was a strong predictor of EA (bowel length >15% – EA 80% vs bowel length ≤15% – EA 17%, p = 0.008). A diagnosis of gastroschisis showed a negative non-significant correlation with the ability to achieve EA (25% vs 60%, p = 0.12). Overall survival rate was 96%.
AGIR surgery is an important tool in the multidisciplinary management of children with SBS. Percentage of expected small length and aetiology of SBS are likely predictors of achievement of EA in patients undergoing AGIR surgery.
Retrospective Case-Series
Predictors of Intestinal Adaptation in Children
2019, Gastroenterology Clinics of North America
Citation Excerpt :
Gastroschisis, in particular those cases with associated intestinal atresia, has been reported as having a worse prognosis than other congenital malformations. This may be in part due to the associated gastrointestinal dysmotility frequently seen in infants with gastroschisis which presents another hurdle in the successful advancement of enteral feeds.48–50 In the PIFCon analysis, the diagnosis of necrotizing enterocolitis (OR = 2.4), compared with other causes of intestinal failure (IF), was a positive predictor for intestinal adaptation.3,7
Long-term outcomes of ultrashort bowel syndrome due to malrotation with midgut volvulus managed at an interdisciplinary pediatric intestinal rehabilitation center
2019, Journal of Pediatric Surgery
The purpose of this study was to describe long-term outcomes of pediatric-onset ultrashort bowel syndrome owing to midgut volvulus managed at an interdisciplinary intestinal rehabilitation center.
Patients with a history of malrotation and pediatric-onset midgut volvulus causing extensive bowel loss (< 20% residual small bowel length expected for postconception age) and treated between 2010 and 2017 were reviewed. Data are expressed as median (IQR).
Twenty-three patients had midgut volvulus at age 1 (0–21) day leading to 9 (8–12) percent predicted residual bowel length. Eight (35%) had gastroschisis. Follow-up was 8.5 (6.6–12.2) years from volvulus. Five (22%) patients underwent intestinal/multivisceral transplantation, and all achieved enteral autonomy. Eighteen (78%) patients remained transplant-free, 7 of whom achieved enteral autonomy after 718 (682–1030) days of parenteral nutrition. Transplant-free enteral autonomy was achieved by 0/6 patients with gastroschisis, compared to 7/12 without gastroschisis (p = 0.04). For the overall group, 18 (78%) patients had small bowel bacterial overgrowth, and 7 manifested symptomatic D-lactic acidosis. We observed 2 mortalities, one awaiting transplant and one 4 years following transplantation.
Midgut volvulus owing to malrotation with extensive bowel loss is associated with favorable long-term survival. Transplant-free enteral autonomy may be feasible, particularly in the absence of gastroschisis.
Prognosis study.
IIb, retrospective cohort study.
Long term outcomes of intestinal rehabilitation in children with neonatal very short bowel syndrome: Parenteral nutrition or intestinal transplantation
2019, Clinical Nutrition
Citation Excerpt :
Intestinal transplantation should be discussed on a case-by-case basis because, even if it remains a difficult procedure, it can offer successful rehabilitation in carefully selected patients [15,23,37,38]. Results from our study, with seven out of the eight gastroschisis included requiring ITX, confirm gastroschisis as a risk factor for the progression to nutritional failure requiring ITx [39]. Stoma duration seems also to play a role in this progression.
Intestinal rehabilitation is the preferred treatment for children with short bowel syndrome (SBS) whatever the residual bowel length, and depends on the accurate management of long-term parenteral nutrition (PN). If nutritional failure develops, intestinal transplantation (ITx) should be discussed and may be life-saving. This study aimed to evaluate survival, PN dependency and nutritional status in children with neonatal very SBS on PN or after ITx, in order to define indications and timing of both treatments.
This retrospective cross-sectional study enrolled 36 children with very SBS (<40 cm) who entered our intestinal rehabilitation program from 1987 to 2007.
All the children on long-term PN (n = 16) survived with a follow-up of 17 years (9–20). Six of them were eventually weaned off PN. Twenty children underwent ITx: eight children died (40%) 29 months (0–127) after Tx. The others 12 patients were weaned off PN 73 days (13–330) after Tx. Follow-up after transplantation was 14 years (6–28). Seven out of 8 (88%) patients with a history of gastroschisis required ITx. Patients who required ITx had longer stoma duration.
Survival rate of children with very short bowel was excellent if no life-threatening complications requiring transplantation developed. Gastroschisis and delayed ostomy closure are confirmed as risk factor for nutritional failure. Intestinal rehabilitation may allow a total weaning of PN before adulthood. A follow-up by a multidisciplinary team is necessary to avoid PN complications in order to minimize indications for ITx.

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CandidateLong-Term Outcomes of Short Bowel Syndrome Requiring Long-Term/Home Intravenous Nutrition Compared in Children With Gastroschisis and Those With Volvulus

Abstract

Introduction

Methods

Results

Conclusions

Section snippets

Patients and Methods

Results

Discussion

Gastroenterology

Best Pract Res Clin Gastroenterol

J Pediatr Surg

J Pediatr

J Pediatr

J Pediatr Surg

Candidate
Long-Term Outcomes of Short Bowel Syndrome Requiring Long-Term/Home Intravenous Nutrition Compared in Children With Gastroschisis and Those With Volvulus