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

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

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.     

Two cases of living-related intestinal transplantation

We recently performed living-related small bowel transplant in two patients. The first patient was a 14-year-old boy with total parenteral nutrition (TPN)-dependent short-bowel syndrome associated with hypoganglionosis. He received a bowel graft from his 43-year-old mother. The second patient was a 27-year-old female who had undergone massive enterectomy due to volvulus. She underwent living-related bowel transplantation from her 57-year-old mother. In both cases, blood types were ABO identical, cytotoxic cross matches were negative, and cytomegalovirus status was positive to positive in both cases. Up to one third of the donor bowel was harvested from the distal ileum. The graft vessels were connected to infrarenal aorta and inferior vena cava. The immunosuppressive regimen consisted of daclizumab, tacrolimus, and steroids. The first patient developed progressive acute cellular rejection on postoperative day 9, requiring OKT-3 therapy. Two months after transplantation, he was weaned off TPN, tolerating oral intake with a fully functioning graft. The second patient was weaned off TPN on day 29 with a functioning graft. Her metabolic disorder dramatically improved. This patient developed indeterminate acute cellular rejection on day 111, which was successfully treated with bolus injections of steroid. Both donors had no complications; they were discharged on day 10. Living-related intestinal transplantation can be a treatment option for patients with short-bowel syndrome.     

Living-related small bowel transplantation: two cases experiences

We have recently performed living-related small bowel transplantation for 2 patients. The first patient was a 14-year-old boy with total parenteral nutrition (TPN)-dependent short-bowel syndrome associated with hypoganglionosis of the entire intestine. He received a bowel graft from his 43-year-old mother. The second patient was a 27-year-old woman, who had massive enterectomy due to volvulus and developed vitamin deficiencies and severe metabolic disorders as a result of long-term TPN. She underwent living-related bowel transplantation from her 57-year-old mother. Blood types were ABO identical, cytotoxic cross matches were negative, and cytomegalovirus statuses were positive-to-positive in both cases. Up to one third of the donor bowel was harvested from the donor distal ileum more than 30 cm away from the ileocecal valve. The graft vessels were connected to infrarenal aorta, and inferior vena cava. The immunosuppressive regimen consisted of daclizumab, tacrolimus, and steroid. The graft surveillance was accomplished using zoom endoscopy and mucosal biopsy. The first patient developed progressive acute cellular rejection (ACR) on the 9th postoperative day (POD)-9 requiring OKT-3 therapy, which was effective. Two months after transplantation, he was weaned from TPN, tolerating oral intake with a fully functioning graft. The second patient experienced no episode of ACR and was weaned off TPN on POD-29 with a functioning graft. Her metabolic disorder dramatically improved after bowel transplantation. Both donors had no complication and were discharged from the hospital on POD-10. Living-related bowel transplantation is an extreme option of treatment for patients with short-bowel syndrome.     

Long-term parenteral nutrition in pediatric autoimmune enteropathies

Severe and protracted or persistent diarrhea (SPD) is the most severe form of diarrhea in infancy and has also been defined as intractable diarrhea when it leads to dependence on total parenteral nutrition (TPN). One of the rare causes of SPD is represented by autoimmune enteropathy that is characterized by life-threatening diarrhea mainly occurring within the first years of life, persistent villous atrophy in consecutive biopsies, resistance to bowel rest, and evidence of antigut autoantibodies. We evaluated 10 patients (seven boys, mean age at diagnosis 18 months; range: 0 to 160 months) fulfilling criteria of autoimmune enteropathy to assess dependence on TPN. TPN was first required in all patients to avoid dehydration and electrolytic imbalance. All patients were dependent on immunosuppressive therapy (steroid, azothioprine, cyclosporine, tacrolimus). Three patients died of sepsis: two during TPN while in the hospital, and one at home after he was weaned off TPN. Five patients are weaned off TPN after a mean period of 18 months; they are actually on oral alimentation with a cow milk-free diet after a period of enteral nutrition with elemental formula. One underwent total colectomy and bone marrow transplantation and one developed an IPEX syndrome. One patient is still dependent on TPN for 24 months. She is on home parenteral nutrition. Patients with diagnosis of IPEX syndrome require parenteral support with three or four infusion per week. TPN represents a fixed step in the management of autoimmune enteropathy, but it may be considered as an interim treatment while waiting for intestinal adaptation, at least in some selectioned case of autoimmune enteropathy. Bone marrow transplantation should be considered and reserved for those patients with severe complications due to home parenteral nutrition, or in those that are really dependent on parenteral nutrition.     

One-Step Management of Apple-Peel Atresia

Purpose: Apple-peel atresia is a rare form of bowel atresia associating proximal jejunal atresia with a typical coil-shaped distal small bowel. Many of the children suffering from this condition are still managed with initial enterostomy followed by delayed anastomosis.

Patients and method: Between 2000 and 2007, we managed four patients with apple-peel atresia. Three by primary anastomosis and one by initial enterostomy with delayed anastomosis.

Results: Total duration of total parenteral nutrition (TPN) was an average 109 days. Survival rate was 100% with an average follow-up of 4.3 years.

Conclusion: Nowadays, the prognosis of apple-peel atresia depends mainly on prolonged TPN-related morbidity rather than immediate postoperative complications. We therefore recommend that those infants affected be managed by primary anastomosis in order to reduce the duration of parenteral nutrition, followed by careful nutrition by teams experienced in neonatal TPN and short bowel syndrome.     

Colon interposition: an adjuvant operation for short-gut syndrome

Six infants with short-gut syndrome refractory to medical management underwent isoperistaltic colon interposition (length 11.7 +/- 3.1 cm.). The abdominal catastrophes that required extensive intestinal resection were: volvulus (3), necrotizing enterocolitis (2), and gastroschisis with intestinal atresia (1). No infant had another major congenital anomaly. The average trial of attempted medical therapy prior to colon interposition was 5.5 +/- 3.6 months. There was no perioperative mortality or morbidity associated with the colon interposition. Following the colon interposition, three infants were weaned from total parenteral nutrition (TPN) in 3 +/- 1 months and all survived. In contrast, three infants could not be withdrawn from TPN and they died secondary to complications of TPN (2 from sepsis and 1 from hepatic failure). Long-term survival was associated with a greater length of small bowel remaining after the initial resection (51 +/- 12 cm v 35 +/- 24 cm), colon interposition at a younger age (3 +/- 1 months v 8 +/- 3.5 months), and a shorter duration of medical management prior to colon interposition (2.8 +/- 0.8 months v 6.7 +/- 5.0 months). All survivors are now tolerating a regular diet and having one to four formed stools per day. Normal somatic growth and developmental milestones are being achieved. The follow-up period is from 24 to 84 months. Our experience with the colon interposition in the patient with short gut syndrome has led us to conclude that when a reasonable trial of medical management has failed, a colon interposition is a safe and effective adjuvant to treatment.     

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.     

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.     

Management of the short-bowel syndrome

The patient with short-bowel syndrome after massive small-intestinal resection represents one of the greatest clinical challenges a general surgeon must face. Maintaining optimal nutritional and metabolic support until maximum bowel adaptation can occur is the top priority of therapy. Currently, no operative procedure for adjunctive management of the short-bowel syndrome is sufficiently safe and effective to recommend its routine use. Long-term parenteral nutrition remains the cornerstone of successful management.     

Resolution of intractable cholestasis associated with total parenteral nutrition following biliary irrigation

Although the protracted use of total parenteral nutrition (TPN) in infants is known to predispose to cholestasis, which in certain instances may not be reversible, failure to understand the pathogenesis of this condition has hindered the development of an effective medical treatment. That surgical treatment may reverse this process in selected patients is the subject of the present report. Two premature infants and one term infant, with short bowel syndrome acquired in infancy, developed conjugated hyperbilirubinemia following institution of balanced TPN. Infectious and metabolic etiologies for the jaundice were ruled out. No excretion of 99mTc-labeled DISIDA from the biliary tree was demonstrable 24 hours following injection by nuclear scintigraphy, despite the absence of extrahepatic biliary obstruction by abdominal sonography. When all conventional medical methods of managing the problem failed, exploration was performed. Intraoperative cholangiography showed normal intrahepatic and extrahepatic biliary ducts in each instance. Irrigation of the biliary tree with normal saline solution was then performed. In all cases, liver biopsies were obtained that were consistent with TPN-associated cholestasis and in all cases, jaundice resolved promptly following operation. We conclude that intractable cholestasis associated with TPN, regardless of its etiology, is a functional abnormality of biliary excretion that may respond in selected patients to irrigation of the biliary tree with radiographic dye and/or normal saline solution.     

Incidence, prognosis, and etiology of end-stage liver disease in patients receiving home total parenteral nutrition.

BACKGROUND: Elevations in liver function tests have been reported in patients receiving total parenteral nutrition (TPN). The clinical aspects of end-stage liver disease (ESLD) associated with the prolonged use of home TPN have not been fully clarified. In previous series patients with duodenocolostomies appeared to be at higher risk than persons with some jejunum or ileum remaining in situ. METHODS: The records of 42 patients treated with home TPN for more than 1 year were examined. This constituted 283 person-years of home TPN. Patients with duodenocolostomies were examined as a separate group on the basis of the literature experience. RESULTS: Six of 42 patients who received chronic home TPN had ESLD with 100% subsequent mortality, at an average of 10.8 +/- 7.1 months after the initial bilirubin elevation. Thirteen of 42 patients had superior mesenteric artery or vein thrombosis (SMT) leading to duodenocolostomy. In 8 of these 13 patients with SMT and underlying inflammatory or malignant disorder, 2 had ESLD. The remaining 5 SMT patients who had only minimal liver enzyme elevation over 13.6 +/- 6.7 (range 3 to 19) years of home TPN were significantly younger (36 +/- 7 years vs 64 +/- 13 years) and did not have underlying inflammation either by clinical diagnosis or as reflected in the high normal serum albumin level (> or = 4.0 g/dL). Despite their extreme short bowel syndrome and long exposure to home TPN, ESLD did not develop. CONCLUSIONS: Approximately 15% of patients who receive prolonged TPN have ESLD with a high rate of morbidity and mortality. The combination of chronic inflammation and the short bowel syndrome appears to be necessary for the development of ESLD with prolonged home TPN.     

Successful treatment of trauma-induced short bowel syndrome with early living related bowel transplantation

BACKGROUND:: Total parenteral nutrition (TPN) is a life-saving therapy for patients with short bowel syndrome. However, TPN is associated with a high incidence of serious complications, poor quality of life, and elevated cost. An attempt was made to avoid TPN-related complications associated with trauma-induced short bowel syndrome by using early living related donor bowel transplantation. METHODS:: Three men 27 to 30 years of age with trauma-induced short bowel syndrome received early living related donor bowel transplantation using segmental ileal grafts. RESULTS:: All the donors had an uncomplicated postoperative course. After a mean follow-up period of 40 months, all three recipients were alive and well, and did not require any TPN support. The ileal graft adapted perfectly to support fully the nutritional needs of young, active individuals. CONCLUSIONS:: Early living related donor bowel transplantation is a successful treatment for trauma-induced short bowel syndrome. It is associated with a lower incidence of complications, better quality of life, and lower cost than long-term TPN.     

Experience with intestinal lengthening for the short-bowel syndrome

Patients with the short-bowel syndrome frequently develop dilated intestinal segments that may lead to impaired motility and malabsorption. Although intestinal tapering alone improves motility, the intestine can be lengthened as well. We reviewed our experience with six children undergoing intestinal lengthening to improve intestinal absorption secondary to the short-bowel syndrome. The procedure was performed by dissecting the vessels along the mesenteric border and dividing the intestine longitudinally with a stapler. Five patients were receiving total parenteral nutrition (TPN) and one was becoming malnourished with enteral feedings alone. Bacterial overgrowth was documented in four patients and abnormal liver function in three patients. The intestinal segments were dilated up to 10 cm in diameter and remnant length ranged from 15 to 79 cm. Segments 5 to 25 cm in length were divided, resulting in an average increase in length of 52%. Necrosis of one of the divided limbs necessitated resection in one patient. Follow-up ranged from 2 to 84 months. TPN has been discontinued in four patients and avoided in another. Symptomatic improvement occurred in all patients. We feel the tapering and lengthening procedure should be considered in patients with symptomatic, dilated intestinal segments in whom the need for TPN may potentially be obviated.     

A new treatment for patients with short-bowel syndrome. Growth hormone, glutamine, and a modified diet.

OBJECTIVE: The purpose of this study was to initially determine if growth hormone or nutrients, given alone or together, could enhance absorption from the remnant small bowel after massive intestinal resection. If clinical improvement were observed, this therapy would then be used to treat patients with the short-bowel syndrome over the long term. SUMMARY BACKGROUND DATA: Patients who undergo extensive resection of the gastrointestinal tract frequently develop malabsorption and require long-term parenteral nutrition. The authors hypothesized that the administration of growth factors and/or nutrients could enhance further compensation of the remnant intestine and thereby improve absorption. Specifically, animal studies have shown that there is enhanced cellularity with the administration of growth hormone (GH) or glutamine (GLN), or a fiber-containing diet. METHODS: Initially, 17 studies were performed in 15 total parenteral nutrition (TPN)-dependent short-bowel patients over 3 to 4 weeks in the clinical research center; the first week served as a control period, and during the next 1 to 3 weeks, the specific treatment was administered and evaluated. Throughout the study, food of known composition was provided and all stool was collected and analyzed to determine absorption across the remaining bowel. The effect of a high-carbohydrate, low-fat diet (DIET), the amino acid glutamine (GLN) and growth hormone (GH) administered alone or in combination with the other therapies (GH + GLN + DIET) was evaluated. The treatment was expanded to 47 adults (25 men, 22 women) with the short-bowel syndrome, dependent on TPN for 6 +/- 1 years. The average age was 46 +/- 2 years, and the average jejunal-ileal length was 50 +/- 7 cm (median 35 cm) in those with all or a portion of colon and 102 +/- 24 cm (median 102 cm) in those with no colon. After 28 days of therapy, the patients were discharged on only GLN + DIET. RESULTS: The initial balance studies indicated improvement in absorption of protein by 39% accompanied by a 33% decrease in stool output with the GH + GLN + DIET. In the long-term study, 40% of the group remain off TPN and an additional 40% have reduced their TPN requirements, with follow-up averaging a year and the longest being over 5 years. CONCLUSION: GH + GLN + DIET offers a potential method for providing cost-effective rehabilitation of surgical patients who have the short-bowel syndrome or other complex problems of the gastrointestinal tract. This therapeutic combination also may be useful to enhance bowel function in patients with other gastrointestinal diseases and those requiring extensive intestinal operations, including transplantation.     

Therapeutic and diagnostic implications of acquired choledochal obstruction in infancy: spontaneous resolution in three infants

Three infants aged 2 days to 11 weeks with conjugated hyperbilirubinenemia, had sonographically documented dilated common hepatic bile ducts, and echogenic material in the gallbladder. A 2-day-old infant, born to a diabetic mother, had none of the classic predisposing factors for cholelithiasis, and two infants had received total parenteral nutrition (TPN) and TPN plus furosemide. The first infant after receiving 4 1/2 weeks of TPN and furosemide, developed common duct obstruction with increasing bilirubin and hepatic duct caliber over a 12-day period. An operation was scheduled; however, on the following day the bilirubin dropped abruptly and surgery was canceled. The experience with this infant encouraged conservative management in two subsequent infants with similar clinical and sonographic findings. Spontaneous resolution occurred 9 days after the onset of common duct obstruction in one infant and after 16 days in the other.     

Isolated liver transplantation in an infant with ultrashort gut

Intestinal function in children with very short bowel syndrome and related intestinal failure may improve after isolated liver transplantation. An infant with an ultrashort gut, ileo-cecal valve, and whole colon received total parenteral nutrition from the first days of life. Enteral feeding failed because of the progressive dilatation of the jejunal portion and motility disorders. He developed early severe cholestatic liver disease (aspartate transferase 186, alanine transferase 103 U/L, serum bilirubin 8.4 mg/dL) and subsequent liver failure. At 8 months of age, he benefited from isolated liver transplantation (left segment graft from living donor). His early posttransplant evolution was characterized by recovery of oral alimentation, improvement of digestive and absorption functions, but he did not achieve TPN-independence. At 20 months, 50% to 60% of his energy needs were covered by parenteral nutrition and he has satisfactory growth indices (3rd percentile for weight and height), reduced stool volume, and frequency. Isolated liver transplantation allowed, in this particular case, time for further intestinal adaptation thereby avoiding the need for intestinal transplantation early in life.     

Short-bowel syndrome – surgical treatment with long-term benefit?

Survival and management of patients with irreversible intestinal failure has been made possible by the development of total parenteral nutrition (TPN). Despite the progress of TPN severe and even fatal complications might occur. Different non-surgical and surgical therapies can be employed to either improve intestinal function or restore enteral autonomy to obviate the need for TPN. A comprehensive review of the pertinent literature on different treatment modalities for short-bowel syndrome and our own experience are presented to judge long-term benefit and make recommendations on up-to-date surgical management. Received: 5 August 1999 Accepted: 18 October 1999     

Primary anastomosis in apple-peel bowel syndrome

During a 15-year period, 12 infants with apple-peel bowel were treated; the first 4 by initial enterostomy with delayed anastomosis and the remaining 8 by primary anastomosis. All four infants in the enterostomy group required parenteral nutrition, compared with only six of the eight who had a primary anastomosis. Seven of the eight infants treated by primary anastomosis were in hospital for less than 50 days, (however, the remaining child was an in-patient for over 2 years, but did suffer from short-bowel syndrome with only 17 cm of small intestine). In comparison, all four of the enterostomy group were hospitalised for between 70 and 175 days. All twelve patients survived.     

Morbidity and mortality of short-bowel syndrome in infants with abdominal wall defects

Total parenteral nutrition (TPN) has made survival beyond infancy possible for many infants who have sustained small intestinal loss as a result of gastroschisis or omphalocele. The length and quality of life in these patients have often been limited by the development of late sequelae secondary both to the protracted use of TPN and the long-term complications of a shortened gut. This study was undertaken to determine what factors influence the morbidity and mortality of short-bowel syndrome (SBS) due to gastroschisis or omphalocele. A retrospective chart review of 850 infants who received TPN from January 1977 through December 1999 was carried out. All infants were treated at one academic medical center; those who had received > or =3 months of TPN were further segregated and their diagnosis, surgical procedures, length of bowel, ability to wean from TPN, follow-up weight and height, and developmental progress were recorded. Seventeen children were identified with SBS and either gastroschisis or omphalocele. Tight primary or secondary closure of the abdominal wall was believed to be a major cause of bowel necrosis and SBS in at least ten of the 17 patients. Overall survival was 76 per cent (13/17); survival was correlated with length of remaining bowel and was 86 per cent in patients having more than 15 cm of small bowel remaining but only 33 per cent in patients with less than 15 cm of small bowel remaining (P = 0.05). A longer length of residual small bowel resulted in a significantly shorter duration of TPN with a mean duration of 1.0 year for survivors having >38 cm and 10.0 years for survivors with <38 cm of bowel remaining (P = 0.03). Hepatic dysfunction with progressive failure resulting from TPN was related to death in three of the four nonsurvivors. The presence or absence of an ileocecal valve appeared unrelated both to the success of TPN weaning and to the length of time on TPN (P > 0.2). Eight of the 13 survivors have no ileocecal valve; five have undergone >50 per cent colonic resection. Nine of the survivors have adapted to enteral feedings (mean 36 +/- 60 months) during which time weaning from TPN occurred. The mean age of survivors is 7.9 +/- 5.1 years. Near-normal weights (defined as exceeding the fifth percentile for weight) were achieved for 92 per cent of the patients (12/13) with 46 per cent of the patients (6/13) exceeding the 50th percentile. Near-normal heights (exceeding the fifth percentile) were achieved for 77 per cent of the patients (10/13) with 15 per cent of the patients (2/13) exceeding the 50th percentile. Quality of life was measured on the basis of return to public school: nine of ten school-age survivors attend school and eight are normal without signs of developmental delay. Tight abdominal closure of gastroschisis or omphalocele may cause bowel necrosis and SBS. TPN has improved the long-term survival and quality of life in infants with SBS.