The prevention and treatment of intestinal failure-associated liver disease in neonates and children

Intestinal Failure Associated Liver Disease (IFALD) is a common and potentially life-threatening problem for pediatric patients receiving long-term parenteral nutrition (PN). Risk factors for IFALD include premature birth, low birth weight, long-term PN, intestinal stasis and sepsis. Preventative strategies are the cornerstone of improving outcomes in IFALD and include enteral feeding, weaning of PN, reduced dose lipid emulsions and the early recognition and treatment of sepsis. Recent work also demonstrates the efficacy of fish-oil based lipid emulsions in the prevention and treatment of IFALD. Transplantation is an option for end-stage liver disease but is associated with significant morbidity and mortality.     

Reversal of intestinal failure-associated liver disease in infants and children on parenteral nutrition: experience with 93 patients at a referral center for intestinal rehabilitation

Purpose

Intestinal failure (IF)-associated liver disease (IFALD) complicates the treatment of children with IF receiving parenteral nutrition (PN). We hypothesized that prevention or resolution of IFALD was possible in most children and that this would result in improved outcomes.

Methods

We reviewed prospectively gathered data on all children referred to the intestinal rehabilitation and transplantation center at our institution. Total bilirubin level (TB) was used as the marker for IFALD. Patients were grouped based on TB at referral and at subsequent inpatient stays and outpatient visits. Standard treatment consisted of cycling of PN, limiting lipid infusion, enteral stimulation, use of ursodeoxycholic acid, and surgical intervention when necessary. Outcomes such as mortality, dependence on PN, and need for transplantation were assessed. Statistical analyses were performed using Fisher's exact, Mann-Whitney U, and Wilcoxon signed rank tests.

Results

Ninety-three patients with intestinal failure and on PN were treated at our center from 2003 to 2009. Median age at referral was 5 months (0.5-264 months). Prematurity was a complicating factor in 63 patients and necrotizing enterocolitis was the most common diagnosis. Eighty-two children had short bowel syndrome, whereas the remaining 11 had extensive motility disorders. 97% of children required significant alteration of their PN administration. At referral, 76 of 93 children had TB 2.0 mg/dL or higher, and 17 had TB below 2.0 mg/dL. TB normalized in 57 of 76 children with elevated TB at referral, and TB remained elevated in 19. Normalization of TB was associated with a mortality of 5.2%, and transplantation was needed in 5.2%. Conversely, when TB remained elevated, mortality was 58% (P = .0002 vs TB normalized), and transplantation occurred in 58% owing to failure of surgical and medical rehabilitation.

Conclusions

Most children referred for treatment of IF have IFALD. A dedicated IF rehabilitation program can reverse IFALD in many children, and this is associated with improved outcome.     

Intestinal failure-associated liver disease in surgical infants requiring long-term parenteral nutrition

PurposeOur aim was to determine incidence, severity, and outcome, as well as predisposing factors and underlying diagnoses, of intestinal failure-associated liver disease (IFALD) in surgical infants requiring long-term parenteral nutrition (PN).MethodsWe retrospectively studied surgical infants receiving PN for at least 28 days for congenital or acquired intestinal anomalies over a 5-year period (January 2006 to December 2010). Intestinal failure-associated liver disease was defined as type 1 (early)—persistent elevation of alkaline phosphatase for 6 weeks or longer; type 2 (established)—additional elevated total bilirubin (≥50 μmol/L); and type 3 (late)—additional clinical signs of end-stage liver disease.ResultsEighty-seven infants required PN for at least 28 days. Intestinal failure-associated liver disease occurred in 29 infants (33%). Intestinal failure-associated liver disease was managed medically in all but 2 patients who underwent intestinal elongation. None were referred for intestinal or liver transplant. Intestinal failure-associated liver disease has been reversed in 17 (59%) of cases to date. Sixty-one children receiving long-term PN (70%) have achieved enteral autonomy, whereas 12 (14%) require home PN. Severity of IFALD was significantly associated with duration of PN and female sex.ConclusionIntestinal failure-associated liver disease remains a fairly common but rarely life-threatening complication of intestinal failure in surgical infants. Intestinal failure-associated liver disease can be reversed in more than half of these children, and enteral autonomy was achieved in more than two thirds, even with minimal use of intestinal elongation. This is the first study to demonstrate an association between the severity of IFALD in surgical infants and female sex.     

Visceral transplantation in patients with intestinal‐failure associated liver disease: Evolving indications,graft selection,and outcomes

Intestinal failure (IF)‐associated liver disease (IFALD) is widely recognized as a lethal complication of long‐term parenteral nutrition. The pathophysiology of IFALD is poorly understood but appears to be multifactorial and related to the inflammatory state in the patient with IF. Visceral transplant for IFALD includes variants of intestine, liver, or combined liver–intestine allografts. Graft selection for an individual patient depends on the etiology of IF, abdominal and vascular anatomy, severity of IFALD, and potential for intestinal rehabilitation. The past decade has witnessed dramatic improvement in the management of IFALD, principally due to improved lipid emulsion formulations and the multidisciplinary care of the patient with IF. As the recognition and treatment of IFALD continue to improve, the requirement of liver‐inclusive visceral grafts appears to be decreasing, representing a paradigm shift in the care of the patient with IF. This review highlights the current indications, graft selection, and outcomes of visceral transplantation for IFALD.     

Isolated liver transplantation in infants with end-stage liver disease associated with short bowel syndrome

OBJECTIVE: To evaluate experience with isolated orthotopic liver transplantation in children with liver failure associated with short bowel syndrome (SBS). SUMMARY BACKGROUND DATA: Infants who have liver failure as a result of SBS are frequently referred for consideration for combined liver and small bowel transplantation. In a few patients the liver disease develops despite a seemingly adequate bowel, which if given time and appropriate management has the potential for full enteral adaptation. There is a limited literature suggesting the utility of OLT without replacement of the native bowel. The advantages over combined liver and small bowel transplantation are clear: organ availability is greater, liver-reduction techniques are well established, lower immunosuppression is required, and there is greater experience in the care of children after orthotopic liver transplantation. METHODS: Eleven infants, considered to have a good prospect of eventual gut adaptation to full enteral nutrition if it were not for their advanced liver disease, underwent isolated orthotopic liver transplantation. Age range was 6.5 to 17.7 months. All patients had been dependent on parenteral feeding but had also shown significant enteral tolerance at some time before listing for transplantation. Advanced liver disease was apparent both clinically and on histologic examination. All were jaundiced and had low albumin levels, and most had coagulopathy. As a group the infants had growth retardation. Estimated remaining length of small bowel beyond the ligament of Treitz was in the range of 25 to more than 100 cm. Six infants retained their ileocecal valve. RESULTS: Thirteen liver transplants were performed in the 11 patients. A combination of whole livers (n = 6) and reduced-size grafts, of which three were from living-related donors, were used. Biliary anastomosis was duct-to-duct in eight instances and involved a short Roux limb in the others. Eight patients are alive with follow-up of 15 to 66 months. Three deaths have occurred after transplantation as a result of sepsis. Of eight surviving patients, only two continue to receive intravenous support and in both there is increasing enteral tolerance. Since transplantation, all surviving children have shown adequate growth with maintenance of pretransplant centiles. CONCLUSIONS: In selected infants with liver failure secondary to short bowel syndrome in whom complete enteral autonomy is anticipated, isolated liver transplantation can offer long-term survival.     

Prevalence of life-threatening complications in pediatric patients affected by intestinal failure

Intestinal failure (IF) is defined as the reduction of functional gut mass necessary to maintain health and growth in children. Causes of IF include short bowel syndrome (SBS), neuromuscular intestinal disorders (NID), and severe protracted diarrhea (SPD). If patients require long-term parenteral nutrition (PN); they can now be discharged on home PN (HPN), thus improving their quality of life. Children requiring long-term PN are at high risk of developing life-threatening IF complications that hinder HPN, namely, IF associated liver disease (IFALD), catheter-related infections (CRI), and thrombosis. The goal of our study was to retrospectively evaluate the prevalence of life-threatening complications among IF patients according to the HPN indication. From January 1989 to May 2006, 60 IF patients (41 boys and 19 girls) underwent prolonged HPN. Total program duration was 46,391 days (127 total years, mean 2.1 years per patient). Indications for HPN were SBS in 36 cases, SPD in 19 cases, or NID in 5 cases. In our experience patients affected by SBS displayed a significantly higher prevalence of life-threatening complications than patients with other IF causes. Sixteen (27%) among 60 patients developed IFALD. CRI and thrombosis prevalence were 1.4/1000 central venous catheter (CVC) days and 0.2/1000 CVC days respectively. SBS seemed to lead to life-threatening complications more often than other HPN indications. SBS patients on long-term PN therefore require careful management to identify complications early, and they seem to be the candidates for early referral to small bowel transplantation centers.     

Isoperistaltic bowel lengthening for short bowel syndrome in children

BACKGROUND: Short bowel syndrome, secondary to a variety of causes, can be lethal in infancy and childhood. Isoperistaltic bowel lengthening, performed by longitudinal division of dilated small bowel with end-to-end anastomosis, has shown early promise but long-term outcome is unknown. METHODS: Sixteen infants and children (aged 3 months to 14 years) had short bowel syndrome from necrotizing enterocolitis (8), gastroschisis (4), atresia (2), and volvulus (2). All of these patients were partially or totally dependent on parenteral nutrition and have undergone isoperistaltic bowel lengthening for short bowel syndrome (length <100 cm). Bowel length was increased by 22% to 85% (mean 42%) with the procedure. Studies of intestinal function were performed preoperatively and postoperatively. RESULTS: Isoperistaltic bowel lengthening resulted in significant improvement in stool counts, intestinal transmit time, intestinal clearance of barium, D-xylose absorption, and fat absorption at 6 months and 12 months postoperatively. Fourteen of 16 patients (88%) have been weaned from parenteral nutrition. CONCLUSIONS: These data show that isoperistaltic bowel lengthening can be an effective operation for short bowel syndrome in children, improving absorption and motility, and allowing weaning from parenteral nutrition.     

Functional liver recovery parallels autologous gut salvage in short bowel syndrome

Background/purpose

Parenteral nutrition (PN) is life saving in short bowel syndrome. However, long-term parenteral nutrition is frequently complicated by a syndrome of progressive cholestatic liver disease that is considered to be irreversible beyond the early stages of cholestasis, particularly in the presence of any degree of fibrosis in the liver. The purpose of this study was to examine apparent improvement in PN-associated liver dysfunction in a cohort of children with short bowel syndrome.

Methods

A retrospective case-record review of all patients managed within a dedicated Intestinal Rehabilitation Program (IRP) identified 13 patients with short bowel who had PN-associated liver dysfunction, defined for this purpose as hyperbilirubinemia or an abnormal liver biopsy.

Results

At referral, 12 of the 13 patients were exclusively on PN, and one was on 50% PN. At current follow-up, 3 patients have achieved complete enteral autonomy from PN, and 7 patients have had smaller decrements in PN requirements. Specific operative procedures to improve intestinal function were undertaken in 11 patients; 4 patients also underwent cholecystectomies with biliary irrigation at the time of intestinal reconstruction. The median highest bilirubin level in these 13 patients was 10.7 mg% (range, 3.2 to 24.5 mg%). Liver biopsy results indicated that 5 patients were cirrhotic, 3 had bridging fibrosis, and 4 had severe cholestasis or lesser degrees of fibrosis. Of 10 survivors in this series, 9 patients currently have a serum bilirubin less than 1 mg% with a median bilirubin in the group of 0.6 mg% (range, 0.3 to 6.4 mg%). Twelve of the 13 patients in this series were initially referred for liver-small bowel transplantation.

Conclusions

This preliminary experience suggests that PN-dependent patients with advanced liver dysfunction in the setting of the short bowel syndrome may, in some instances, experience functional and biochemical liver recovery. The latter appears to parallel autologous gut salvage in most cases. As a corollary, the authors believe that even advanced degrees of liver dysfunction should not preclude attempts at autologous gut salvage in very carefully selected patients. Such a policy of “aggressive conservatism” may help avoid the need for liver/intestinal transplantation in some patients who appear to be not responding to PN.     

Lethal short-bowel syndrome

Infants with short-bowel syndrome are difficult to manage. Despite supportive measures with parenteral nutrition and surgery to lengthen remaining bowel or increase functional absorptive surface area, the outcome for many of these infants is poor. We have reviewed a series of seven infants diagnosed with severe short bowel. Causes included volvulus (3), multiple atresias (2), and total intestinal aganglionosis (2). Survival time ranged from 15 days to 8 months. During the hospital course, each infant underwent one to three operative procedures to diagnose and manage the short bowel and all received total parenteral nutrition (TPN) ranging from 10 days to 6 months. One infant died of liver failure and two others developed significant liver dysfunction secondary to TPN. Most infants remained hospitalized until their death. Death occurred at an average of 9 weeks following the diagnosis of short-bowel syndrome. This review suggests that infants with less than 6 cm of small bowel beyond the Ligament of Treitz will inevitably die of their disease or treatment complications. Until bowel transplant becomes a viable alternative, operative intervention and nutritional support may prolong survival but will not change the outcome of these infants and will only contribute to additional morbidity. A decision to withhold further therapy would be reasonable at the time the diagnosis is established.     

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.     

Isolated liver transplant and sequential small bowel transplantation for intestinal failure and related liver disease in children

Liver dysfunction is a well-recognized complication of intestinal failure in children. Advances in total parenteral nutrition (TPN) have allowed these children to survive while their intestinal tract gradually adapts. Unfortunately TPN may lead to cholestatic liver disease particularly in the young children. Progression of liver disease is associated with a poor prognosis and is an indication for small bowel transplantation.We report our experience of orthotopic liver transplantation in four children with short gut and sequential liver and small bowel transplantation in one child. All children had TPN-related liver failure. Causes of intestinal failure included necrotising enterocolitis (n=2), gastroschisis (n=1), intestinal atresia (n=1), and megacystic, microcolon syndrome (n=1). At the time of liver transplantation the children's mean age was 10.9 months (2.5-24) and weight 6.7 kg (4.8-10.1). The mean serum bilirubin was 522 micromol/liter (299-823), aspartate transaminase 423 IU/liter (49-1024) and international normalized ratio 2.8 (2-3.9). There were two deaths both from respiratory failure secondary to adenovirus pneumonia including the child who received a sequential small bowel transplant. Three children with isolated liver grafts are alive and off TPN at 20 months (mean) follow up (range 6-35). Isolated orthotopic liver transplantation has a role in selected children with intestinal failure, particularly those with short but normally functioning gut and progressing with satisfactory intestinal adaptation but developing liver disease. Those children with TPN-related liver disease and unadapted gut or irreversible intestinal disease need combined liver and small bowel transplantation. Sequential small bowel transplantation is feasible after orthotopic liver transplantation and may provide an option for the child with terminal liver and small bowel failure.     

Segmental reversal of the small bowel as an alternative to intestinal transplantation in patients with short bowel syndrome.

OBJECTIVE: This article reports the results of segmental reversal of the small bowel on parenteral nutrition dependency in patients with very short bowel syndrome. SUMMARY BACKGROUND DATA: Segmental reversal of the small bowel could be seen as an acceptable alternative to intestinal transplantation in patients with very short bowel syndrome deemed to be dependent on home parenteral nutrition. METHODS: Eight patients with short bowel syndrome underwent, at the time of intestinal continuity restoration, a segmental reversal of the distal (n = 7) or proximal (n = 1) small bowel. The median length of the remnant small bowel was 40 cm (range, 25 to 70 cm), including a median length of reversed segment of 12 cm (range, 8 to 15 cm). Five patients presented with jejunotransverse anastomosis, and one each with jejunorectal, jejuno left colonic, or jejunocaecal anastomosis with left colostomy. RESULTS: There were no postoperative deaths. Three patients were reoperated early for wound dehiscence, acute cholecystitis, and sepsis of unknown origin. Three patients experienced transient intestinal obstruction, which was treated conservatively. Median follow-up was 35 months (range, 2 to 108 months). One patient died of pulmonary embolism 7 months postoperatively. By the end of follow-up, three patients were on 100% oral nutrition, one had fluid and electrolyte infusions only, and, in the four other patients, parenteral nutrition regimen was reduced to four (range of 3 to 5) cyclic nocturnal infusions per week. Parenteral nutrition cessation was obtained in 3 of 5 patients at 1 years and in 3 of 3 patients at 4 years. CONCLUSION: Segmental reversal of the small bowel could be proposed as an alternative to intestinal transplantation in patients with short bowel syndrome before the possible occurrence of parenteral nutrition-related complications, because weaning for parenteral nutrition (four patients) or reduction of the frequency of infusions (four patients) was observed in the current study.     

Congenital multiple intestinal atresia successfully treated with multiple anastomoses in a premature neonate: Report of a case

We report herein the case of a 2080-g premature male infant born at 34 weeks' gestation with multiple intestinal atresia, for whom multiple anastomoses were successfully performed. A total of 11 atresias were found in the small bowel, and five anastomoses were performed to preserve 59cm of small bowel and the ileocecal valve. Postoperatively, he developed several episodes of sepsis caused by persistent enterostasis, but was able to be weaned from total parenteral nutrition (TPN) by postoperative day (POD) 106. Thus, multiple anastomoses may be the appropriate procedure to prevent short-gut syndrome for congenital multiple intestinal atresia, even in premature infants.     

Peripheral total parenteral nutrition employing a lipid emulsion (Intralipid): complications encountered in pediatric patients.

The clinical records of 180 pediatric patients who received Intralipid via peripheral veins at a single institution (1964-1977) were retrospectively analyzed, with particular reference to the complications of this form of therapy. Intralipid was used in a dose range of 2--4 g/kg/day in order to supply 40% of the daily calorie requirements. The patients were neonates, infants, children, and adolescents with a wide range of clinical diagnoses. Local complications associated with Intralipid therapy were minimal. Transient elevations in serum enzyme levels (SGOT, SGPT, and LDH) were observed in 4% of patients, but all of these returned to the normal range after cessation of therapy. Ten patients had histologic evidence of cholestasis, the significance of which is discussed. The lipid emulsion was employed in patients with preexisting hyperbilirubinemia with concomitant resolution of jaundice. Intralipid was administered to patients with known severe thrombocytopenia (secondary to sepsis or myelosuppression) with return of the platelet counts to normal levels during the course of infusion therapy. The use of Intralipid in patients with established sepsis did not delay its response to conventional surgical or antibiotic therapy. There were no instances of the "overloading" syndrome observed.     

The effect of intravenous lipid emulsions and mucosal adaptation following massive bowel resection

Aims and objectives

Fish oil (FO) lipid emulsion and a new lipid emulsion (SMOF) are important treatments for intestinal failure-associated liver disease. We evaluated the efficacy of FO and SMOF lipid emulsion on intestinal mucosal adaptation using a total parenteral nutrition (TPN)-supported rat model of short bowel syndrome.

Adverse effects of liver dysfunction and portal hypertension on intestinal adaptation in short bowel syndrome in children

BACKGROUND: The effects of liver dysfunction and portal hypertension on intestinal adaptation in short bowel syndrome are generally unknown. The presence of these disorders may adversely affect the ability to wean these patients from parenteral nutrition. METHODS: Forty-two infants with short bowel syndrome were placed in one of three Child's classifications, depending on serum bilirubin, prothrombin time, ascites, albumin, and liver biopsy, and compared for time to diet tolerance, time required for parenteral nutrition, and survival. A subgroup of these patients also underwent portal pressure measurement, which was combined with liver biopsy results to compare three groups for the same parameters. RESULTS: Survival was Child's class A 100%, B 84%, C 61%, while time to feeding tolerance was A 16.3 days, B 20.0 days, C 28 days, and total parenteral nutrition time was A 80.0 days, B 98.0 days, C 100.0 days. In the groups that underwent portal pressure measurement, the survival was group I (normal biopsy and pressure) 100%, group II (abnormal biopsy and normal pressure) 90%, group III (abnormal biopsy and pressure) 66%, while time to feeding tolerance was I 15.0 days, II 18.0 days, III 24.0 days, and total parenteral nutrition time was I 72.0 days, II 94.0 days, III 184.0 days. CONCLUSIONS: Cholestatic liver disease, especially associated with portal hypertension adversely affects bowel adaptation in short bowel syndrome.     

Surgical treatment and problem of massive small intestinal resection in children--assessment of background diseases and actual management

From 1971 to 1986, massive small intestinal resection was done in twenty-five cases; intestinal atresia 12, intestinal volvulus 9, necrotizing enterocolitis 2, intussusception 1 and gastroschisis 1. Thirteen cases (52%) of them have survived. Of 12 cases with intestinal atresia undergoing small intestinal resection, 7 cases had atresias of multiple type, on the other hand, in intestinal volvulus, 4 of 9 cases without malrotation have had massive small intestinal resection, compared with 5 of 30 cases with malrotation. Many clinical problems have occurred after massive small intestinal resection, especially in cases with short bowel syndrome (shorter than 30 cm in length), but home parenteral nutrition has become one of the key treatments for cases with short bowel syndrome.     

Relationship between biopsy-proven parenteralnutrition-associated liver fibrosis and biochemical cholestasis in children with short bowel syndrome

Purpose

The aim of the study was to determine the frequency of biochemical cholestasis (direct bilirubin [DB] ≥2 mg/dL) in children with short bowel syndrome and biopsy-proven parenteral nutrition (PN)-associated liver disease and to define predictive factors for the occurrence and degree of hepatic fibrosis.

Methods

After institutional review board approval, a retrospective review was conducted of patients followed by 2 multidisciplinary intestinal rehabilitation programs between January 1, 2000, and September 30, 2008. Inclusion criteria were exposure to PN (>30 days) and having undergone a liver biopsy. Liver biopsy specimens were graded from 0 to 3 based upon degree of fibrosis in the pathology report. The most recent DB within 10 days before biopsy was recorded.

Results

A total of 66 children underwent 83 liver biopsy procedures. The most common diagnoses included necrotizing enterocolitis (NEC) (36.4%), gastroschisis (22.7%), and intestinal atresia (15.1%). Median age at biopsy was 6.1 months with a median duration of PN of 4.7 months. Of the patients, 70.3% had a history of exposure to parenteral ω-3 lipid emulsion. Of the liver biopsy specimens, 89% (74/83) demonstrated some degree of fibrosis (fibrosis scale 1-3), including 9.6% (8/83) with evidence of cirrhosis. 83% of biopsies without fibrosis and 55% of biopsies with fibrosis were obtained in patients without evidence of biochemical cholestasis (P = .20). Three (37%) of the 8 patients with cirrhosis on liver biopsy had no evidence of biochemical cholestasis. Univariate analysis identified only gestational age (GA) at birth as significantly associated with the degree of liver fibrosis (P = .03). A multivariate logistic regression model accounting for multiple biopsy procedures in patients revealed that GA was a predictor of fibrosis only in patients with a diagnosis other than NEC (P < .01).

Conclusions

In children with short bowel syndrome, biochemical cholestasis does not reflect the presence or degree of histologically confirmed PN-associated liver fibrosis. Careful follow-up, combined with further refinement of diagnostic and hepatoprotective strategies, may be warranted in this patient population.     

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.