Near-total intestinal aganglionosis: long-term follow-up of a morbid condition

PURPOSE: The aim of this study was to understand the long-term outcome of near-total intestinal aganglionosis (NTIA). METHODS: The study was an institutional review of all patients with NTIA between 1974 and 1999, inc!usive. Seven full-term babies were identified (4 boys, 3 girls). Mean birth weight was 3.2 kg (range, 2.7 to 4.1). Patients included 1 pair of siblings and 1 Down's syndrome baby. All 7 had obstruction within 5 days of life. One patient experienced bowel perforation. All 7 had NTIA diagnosed within the first 2 months of life. In 2 of 7, aganglionosis began 10 cm distal to the ligament of Treitz (LOT). The longest segment of normal small bowel was 130 cm from the LOT. Five of 7 babies underwent a stoma between day of life (DOL) 1 and 15. RESULTS: Two of three "long-term" survivors (>2 years) had corrective surgery by 13 months. The first received extended jejuno-ileal myotomy and myectomy with multiple postoperative problems. She died at 8 years of age of complications of total parenteral nutrition (TPN). The second underwent myotomy, resection and patch graft to jejunum, and several jejunostomy revisions. All of the involved bowel eventually was resected. The 3 of 7 who lived more than 1 year all took some nutrition orally. Three died, between 3 months and 8 years of age, of complications of TPN. There are 2 survivors at 3 and 7 years of age. Survival has not correlated with the length of aganglionic bowel. CONCLUSIONS: Aganglionosis involving most of the bowel has a high morbidity and mortality rate. Since 1990 a more aggressive surgical approach has resulted in improved survival rates but with significant morbidity. For children surviving beyond 3 months of age, outcome was less dismal. Some patients may benefit from extended jejunal myotomy or myectomy. However, postoperative complications are the rule, not the exception.     

Proximal jejunostomy with or without myectomy-myotomy modification in five infants with total intestinal aganglionosis: An experience with surgical treatments in a single institution

Background: Total intestinal aganglionosis is characterized by the absence of intramural ganglion cells, in which the disease's involvement extends from the stomach to the anorectum. This disease was suggested previously to be incompatible with life, but recently an extended small bowel myectomy-myotomy has achieved some prolonged survivors. Methods: Five patients with total intestinal aganglionosis underwent laparotomy at 1 to 5 days of age. Surgery was performed as a simple jejunostomy 60 to 70 cm below the ligament of Treitz in the initial 2, jejunustomy 30 cm below the ligament of Treitz in 1, and jejunostomy with myectomy-myotomy modification 30 to 35 cm below the ligament of Treitz in the remaining 2 infants. Results: The initial 2 patients died of sepsis, possibly derived from frequent enteritis and bacterial translocation at 7 and 8 months of age. Another patient had prolonged survival but died of hepatic failure at 1 year, 4 months. The remaining 2 children have survived beyond 2 years of age without any liver dysfunction, receiving a combination of enteral and parenteral nutrition. Conclusions: The more proximal site (30 to 35 cm below the ligament of Treitz) of jejunostomy with myectomy-myotomy modification appeared to be preferable for prolonged survival in these 5 patients with total intestinal aganglionosis. J Pediatr Surg 37:835-839.     

A case report of a patient with near total intestinal aganglionosis followed by the role of extended myectomy and synbiotics therapy

We report our experience with a patient with near-total intestinal aganglionosis who underwent extended myectomy-myotomy of the proximal jejunum. At 45 days of age, the patient underwent rejejunostomy at 34 cm from the ligament of Treiz (LOT) because of enteritis and the bowel rehabilitation therapy was started. Because the patient established the swelling and eating enough at 8 months of age, an extended myectomy-myotomy was performed when she was 245 days old. A 12-cm myectomy from the jejunostomy located at 27 cm from the LOT was coupled with a 30-cm antemesenteric border myotomy. This ultimately left the patient with 65 to 70 cm of small bowel from the LOT. Synbiotics therapy was performed to prevent bacterial translocation, and then central line infection or enteritis was not observed at all. The patient was doing well, weighing 9.65 kg, at 2 years of age. In conclusion, extended myectomy-myotomy has extended the patient's survival period, but it is important and necessary to prevent bacterial translocation and to improve intestinal adaptation by synbiotics therapy.     

Extended myectomy-myotomy. A therapeutic alternative for total intestinal aganglionosis.

OBJECTIVE: That total intestinal aganglionosis (extended Hirschsprung's disease) is uniformly incompatible with life as reported in 1985, is challenged by this series of patients treated over the last 7 years with an alternative therapy, extended myectomy-myotomy of the small bowel. SUMMARY BACKGROUND DATA: A total of 16 neonates worldwide presented with intestinal obstruction secondary to total (extending to the stomach) or near total (to 40 cm of jejunum) intestinal aganglionosis confirmed at one or more leveling operations. METHODS: A patient questionnaire was answered by the surgeon of all 16 patients. RESULTS: The sex distribution was eight boys and eight girls. The definitive operation included extending an antimesenteric myectomy-myotomy from the ganglionic-aganglionic transition zone for variable lengths, the operative design being to create sufficient small bowel length to support life (40-cm minimum, total small bowel maximum). The myectomized bowel was terminated as an end-stroma or as an isolated jejuno-ileal segment. Ten of 16 patients have survived (62.5%) whose length of ganglionated bowel varies from 0 to 40 cm (mean, 12.4 cm; median, 6.0 cm). Six patients have died from 1 to 33 months after operation (mean, 9.5 months; median, 5.0 months) of gut-induced infection (n = 5) and respiratory failure (n = 1); their ganglionated bowel length was similar to survivors (range, 0-26 cm; mean, 9.2 cm; median, 8.0 cm). Of the 16 patients, 15 have received enteral nutrients through the myectomized bowel. Of ten survivors, strikingly two are totally gut nourished (2 cm, 7-cm length of ganglionated bowel), six receive from 1/5 to 4/5 of total calories enterally, and one receives minimal enteral feeding. CONCLUSIONS: From these patients we have learned that (1) extended myectomy-myotomy relieves the obstruction of extended Hirschsprung's disease; (2) aganglionic bowel after extended myectomy-myotomy acts as a passive conduit for proximally propulsed nutrients; and (3) aganglionic bowel after extended myectomy-myotomy undergoes adaptive change and is capable of absorbing life-supporting nutrients. These data demonstrate extended myectomy-myotomy to be a therapeutic option for otherwise fatal extended Hirschsprung's disease, either as a potentially definitive therapy or as a putative bridge to intestinal transplantation.     

Short bowel syndrome in infancy and childhood. Analysis of survival in 60 patients

This report concerns 60 infants and children with short bowel syndrome, most commonly caused by necrotizing enterocolitis in this study. Resection of atretic or gangrenous bowel was performed in 53 patients, tapering enteroplasty and primary anastomosis was performed in 13 patients, and temporary enterostomies were performed in 40 patients. Second-look laparotomy was useful in two of four cases of questionable bowel viability. The ileocecal valve was resected in 33 patients and remained intact in 27. The mean length of remaining bowel was 58.4 cm (range 13 to 150 cm). Seven patients with total aganglionosis and mid to proximal small bowel extension were managed with an initial enterostomy, whereas three had a pull-through procedure with an aganglionic patch enteroplasty. All patients received total parenteral nutrition and early enteral feedings. Home hyperalimentation was attempted when 50 percent of the calorie intake was enteral. Intestinal adaptation required from 3 to 14 months. Frequent setbacks were related to catheter sepsis, rotavirus infection, carbohydrate intolerance, and liver dysfunction. The overall survival rate was 85 percent, with mortality due to liver failure and sepsis associated with total parenteral nutrition.     

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.     

Improved tolerance for enteral nutrition after serial transverse enteroplasty (STEP) in infants and children with short bowel syndrome—A seven-year single-center experience

BackgroundSerial transverse enteroplasty (STEP) was designed to lengthen and taper the small intestine in patients with short bowel syndrome (SBS) and dilated small bowel. We hypothesized that tolerance for enteral nutrition (EN) improves after STEP.MethodsPatients who underwent STEP between March 2004 and January 2011 were identified. Candidates for STEP had radiographic evidence of dilated small bowel and either failed to advance EN or demonstrated deterioration in tolerance for EN. Clinical and nutritional data were analyzed pre- and post-STEP. EN was defined as the percentage of calories administered enterally. Statistical analysis employed the signed rank test with significance assumed when p < 0.05.ResultsTwenty STEPs were performed at a median age of 13.7 months. Median pre-STEP bowel length was 30 cm with a median increase in bowel length of 42%. Five patients achieved enteral autonomy at a median of 6.5 months post-STEP. EN increased in 75%, while 25% exhibited unchanged or decreased EN post-STEP. In aggregate, median EN tolerance increased from 22% at one month pre-STEP to 61% at six months post-STEP (p = 0.003).ConclusionsThe STEP is an effective adjunct in the treatment of patients with intestinal failure. While enteral autonomy is eventually possible in some patients, improved enteral tolerance can be achieved in a majority of cases.     

Extensive intestinal aganglionosis

Extensive aganglionosis very close to the ligament of Treitz and total intestinal aganglionosis are rare forms of Hirschsprung's disease. In these cases, nutrition, fluid, and electrolyte balance are a problem. Although the myectomy-myotomy technique of Ziegler appears hopeful, no effective surgical method has been put into the practice for the treatment of these rare forms of Hirschsprung's disease. We report four cases with extensive intestinal aganglionosis, one of which was total intestinal aganglionosis with involvement of the stomach.     

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.     

Y a-t-il une vie (possible) après une résection intestinale étendue ?

Salvation of patients withmesenteric ischemia often leads to large small bowel resections. Most physicians think that such extensive resection is not reasonable and not compatible with short and long-term survival. However, it is well documented that short bowel syndrome is a condition in which long-term survival is possible. It has been shown that 5-year survival of patients with postischemic short bowel syndrome is about 60%. Factors associated with poor prognosis were end enterostomy, small bowel length shorter than 50 cm and age greater than 60 years. Parenteral nutrition (PN) is the treatment of choice of patients with short bowel syndrom. This PN dependance is related negatively to postduodenal small bowel length (<100 cm), the absence of terminal ileum or colon in continuity. Oral or enteral nutrition should be associated with PN in patients surviving to mesenteric ischemia. Because of adaptative capacity of energy absorption of the colon, the colon should be retablished in continuity each time possible.     

Extreme short-bowel syndrome in an infant

This full term male infant required a 90% enterectomy for congenital intestinal volvulus shortly after birth. The remaining small bowel consisted of 8 cm of proximal jejunum anastomosed to 5 cm of terminal ileum. The ileocecal valve was intact. Parenteral nutrition was administered for the first 21 months, including 17 months of home parenteral nutrition. The child's oral diet was mother's milk until 28 months of age when he was switched to an elemental diet. Solids were introduced at 6 months of age. Presently, at 36 months of age, the infant is off parenteral nutrition and is maintaining good nutritional status on a diet of 150 calories per kilo per day. His major problem has been an extreme allergy to cow's-milk protein. This infant is remarkable, since, despite record short bowel length, he maintained a completely normal growth and development, had remarkably few serious complications, spent relatively little time in hospital, and has documented improvement of G.I. absorption and radiographic contrast studies.     

Massive mesenteric ischemia resulting from a giant strangulated umbilical hernia

Hernia repair is the most commonly practiced operation in the departments of surgery in developed countries. Huge abdominal hernias are uncommon in western civilization. We present a rare case of a 73-year-old woman with a diagnosis at admission of intestinal obstruction caused by a giant strangulated umbilical hernia. At the clinical and radiological examination, the patient showed an enormous strangulated umbilical hernia with acute abdomen, atrial fibrillation, and pulmonary subedema. Emergency laparotomy showed a huge peritoneal umbilical sac containing massive mesenteric ischemia starting from 40 cm after the Treitz ligament and extended to the right colonic flexure. A near-total resection of the small bowel, a right colectomy with double terminal stomas, and a direct hernia repair without prosthetic mesh were performed. Twenty days after the operation, the patient was discharged and begun domiciliary total parenteral nutrition, and 24 months after surgical treatment she is still alive.     

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.     

Triple tube therapy: a novel enteral feeding technique for short bowel syndrome in low-income countries

Background/Purpose

Short bowel syndrome (SBS) is a global malabsorption syndrome that results from extensive intestinal resection. We report our experience of 10 neonates with short bowel managed by a novel triple tube enteral feeding technique. The aim of this research was to provide justification for enteral nutritional strategies to enhance intestinal adaptation, especially in developing countries where parenteral nutrition is either unavailable or expensive.

Methods

From March 2004 to January 2006, 10 neonates underwent extensive bowel resection, enterostomy, and mucous fistula for necrotizing enterocolitis or midgut volvulus and were managed postoperatively with triple tube enteral feeding technique. Gestational age, birth weight, primary abdominal pathology, timing of surgery, surgical procedure performed, complications, duration and feasibility of refeeding, and weight gain were recorded.

Results

The group was composed of 8 male and 2 female neonates with a mean gestational age of 34.2 ± 4.6 weeks and mean birth weight of 2580 ± 993 g. Necrotizing enterocolitis accounted for 7 (70%) and midgut volvulus accounted for 3 (30%) cases of SBS. Mean gestational age at surgery was 35.5 ± 2.2 weeks. Mean residual small bowel length and colon length after resection were 35.5 ± 3.5 and 30.5 ± 1.5 cm, respectively. Weight gain during refeeding ranged from 3 to 6 g/kg per day with duration of refeeding lasting 20 to 156 days. Reanastomoses was done 92 ± 4.2 days after the primary surgery. There were 3 surgery-related complications, but no mortality. Mean period of follow-up was 12 ± 2.4 months. Five of the 10 children are now 19 months old and have a body weight of −1.5 ± 0.64 SD scores and height of −1.75 ± 0.99 SD scores.

Conclusion

This technique represents a safe and effective enteral nutrition strategy that eliminates the need for total parenteral nutrition for SBS in developing countries.     

Small bowel necrosis related to enteral nutrition after duodenal surgery

Nutritional support is the key to the successful recovery of any patient. Small bowel necrosis is described in patients being fed with enteral nutrition after surgery. Five patients with small bowel necrosis after surgery will be discussed and an etiology proposed. A retrospective review of patient data was performed. Data was collected on the type of surgical procedures performed, the enteral nutrition given to the patient, basic laboratory data, the length of stay, and discharge status. A total of five patients' charts were reviewed. Three patients had pancreaticoduodenectomy for a pancreatic mass and two required pyloric exclusion secondary to gunshot wounds. All five patients were fed with a fiber-based enteral nutrition. All patients subsequently had small bowel necrosis requiring reoperation. Four of the five patients had inspissated tube feeding within the necrotic small bowel. Two patients died and three survived with prolonged hospital courses. We propose that the combination of duodenal surgery and fiber-based enteral nutrition contribute to the development of small bowel necrosis postoperatively.     

Do prognostic factors exist for total colonic aganglionosis with ileal involvement?

Background/Purpose: Total colonic aganglionosis with ileal involvement is estimated at 1 case in 50,000 living births. This pathology has a very variable prognosis, and patients often need long-term parenteral nutrition. The aim of this study is to define prognostic factors for this disease. Methods: This is a retrospective study from 1980 to 1999, based on 26 cases of total colonic aganglionosis with ileal involvement. The authors analyzed birth term, sex, birth weight, ileal involvement in centimeters, delay to correct level enterostomy, the total parenteral nutrition duration, and the need for constant rate nutritional assistance. The authors studied the following items: Weight, height, complications, clinical state, and nutritional issues. The statistic test is: LOG RANK (analysis of censured datas and comparison of survival diagram). Results: The only prognostic factor is the length of ileal involvement. All the children with ileal involvement less than 50 cm, except for 2, did not need long-term nutritional assistance; for those with ileal resection over 50 cm, long-term nutritional assistance was needed. Total colonic aganglionosis is a very serious illness (2 children had a small bowel transplantation, and 2 are waiting for one). The prognosis is even worse when associated with a polymalformation syndrome (50% of the children died). Conclusion: The major prognostic factor is the length of small bowel not involved in the total colonic aganglionosis.     

Importance of Ileum and Colon in Children with Short Bowel Syndrome

BackgroundIt is well known that small bowel length is a dominant prognostic indicator in patients with short bowel syndrome (SBS). The relative importance of jejunum, ileum, and colon is less well defined in children with SBS. Here we review the outcome of children with SBS with respect to the type of remnant intestine.MethodsA retrospective review of 51 children with SBS was conducted at a single institution. The duration of parenteral nutrition use was the main outcome variable. The length of the remaining intestine as well as the type of intestine were recorded for each patient. Kaplan–Meier analyses were conducted to compare the subgroups.ResultsChildren with greater than 10% expected small bowel length or more than 30 cm of small bowel achieved enteral autonomy faster than those with less. The presence of ileocecal valve enhanced the ability to wean from parenteral nutrition. The presence of ileum significantly enhanced the ability to wean from parenteral nutrition. Patients with the entire colon also achieved enteral autonomy sooner than those with partial colon.ConclusionsThe preservation of ileum and colon is important in patients with SBS. Approaches to preserve or lengthen ileum and colon may be beneficial for these patients.Level of evidenceIV.     

Waardenburg syndrome with extended aganglionosis: report of 3 new cases

Purpose

The Waardenburg-Shah syndrome is an autosomal recessive disease with varied penetration where Hirschsprung's disease and the Waardenburg syndrome are seen together. Although the length of the involved intestinal segment varies in this syndrome, most patients had total colonic aganglionosis with or without small bowel involvement. We present in this study 2 siblings and one first-degree relative for a total of 3 male patients with Waardenburg syndrome and total colonic aganglionosis with or without small bowel involvement, together with their clinical characteristics and treatment methods.

Patients

The patients who presented with intestinal obstruction findings within the first 48 hours after birth were operated on with 2 patients under elective conditions and 1 as an emergency. The ganglionic segment lengths were 6, 8, and 20 cm, respectively. Aganglionic enterostomy was performed, and the Ziegler operation was used for these patients. The enterostomies started to function on the third postoperative week, and they started to gain weight. However, all died because of sepsis on the 5th to 12th month.

Conclusion

Waardenburg-Shah syndrome patients have a higher incidence of total colonic aganglionosis with or without small bowel involvement. The Ziegler operation may be used in patients with inadequate ganglionic bowel length to gain some time for the child to grow and to decrease total parenteral nutrition complications.     

Isolated liver and multivisceral transplantation for total parenteral nutrition-related end-stage liver disease

Purpose

Total parenteral nutrition (TPN) has prolonged survival in children with intestinal failure; however, end-stage liver disease owing to TPN-induced cholestasis (ESLD-TPN) may preclude its use. ESLD-TPN is an indication for isolated liver transplantation (ILT) or multivisceral transplantation (MVT). Isolated liver transplantation for ESLD-TPN should only be considered in patients who have the potential for enteral autonomy.

Methods

We retrospectively reviewed the records of patients with ESLD-TPN who underwent ILT (n = 7) or MVT (n = 5) between 1994 and 2005. The median age at the time of transplantation was 10.0 months. Intestinal failure followed necrotizing enterocolitis (n = 3), gastroschisis (n = 3), gastroschisis with volvulus (n = 3), gastroschisis with atresia (n = 1), malrotation (n = 1), and megacystis microcolon intestinal hypoperistalsis syndrome (n = 1).

Results

Isolated liver transplant patients had a median length of small bowel of 70 cm and tolerated a median of 50% of enteral calories. The median length of small bowel in patients who underwent MVT was 29 cm, and none tolerated more than 30% of goal enteral feeds. Reduced-size (n = 5) and whole-liver (n = 2) allografts were used for patients undergoing ILT. Patients undergoing MVT received liver-small bowel-pancreas (n = 4) or liver-small bowel-pancreas-colon (n = 1). Overall patient survival was 57% in ILT (median follow-up = 25.1 months); 3 survivors are TPN independent, and the fourth patient requires TPN 3 days/wk. Patient survival was 40% after MVT (median follow-up = 13.0 months); 1 MVT patient died of abuse 16.9 months after transplant and was TPN independent at the time of death. Both survivors are TPN independent. Bilirubin levels are within normal range in all survivors.

Conclusion

Isolated liver transplantation for ESLD-TPN in the setting of intestinal failure is a viable option in patients who have the potential for enteral autonomy. Multivisceral transplantation is the only alternative in patients without the potential for intestinal recovery. Survival can be achieved in patients with ESLD-TPN, but mortality remains high for both procedures.