Enteral Nutritional Support by Percutaneous Endoscopic Gastrostomy in Children with Congenital Heart Disease

One of the major problems of children with severe congenital heart disease (CHD) is their poor nutritional status. Among other consequences, it influences the surgical outcome. Retrospectively we present our experience with percutaneous endoscopic gastrostomy (PEG) in 15 children with CHD. This technique allows enteral nutritional support without the disadvantages related to long-term nasogastric tube feeding. Major complications were absent, and minor complications were rare both at PEG insertion, which was performed under deep sedation, and during feeding via PEG tube. In 4 of the 8 children who were followed for at least 6 months the age-matched body weight increased more than one standard deviation. In 2 other patients it increased more than 0.5 standard deviations. In 7 children the tube was removed after 2.5 to 42 months since enteral support was no longer necessary. Apart from initial reservations the parental acceptance of PEG was good. We conclude that the PEG is a safe and reliable technique to support enteral nutrition in children with severe CHD.     

Body composition of children aged 1 to 12 years with biliary atresia or Alagille syndrome

BACKGROUND: Growth and body composition have not been well described in older children with biliary atresia or Alagille syndrome living with their native liver. To optimize nutritional management of these conditions it is essential to understand the normal growth characteristics. OBJECTIVES: The purpose of this study was to evaluate and compare the growth and body composition of children, particularly school-age children, with biliary atresia and Alagille syndrome. METHODS: A single observer measured height, weight, arm anthropometry and skin fold thickness in subjects aged 1 to 12 years with biliary atresia or Alagille syndrome who had not undergone liver transplantation. RESULTS: Forty-six subjects (10 biliary atresia/36 Alagille syndrome) were assessed. Biliary atresia subjects were below average in height for age with normal weight and elevated body mass index. Mean fat stores were mildly depressed. The z-scores for body mass index and weight for age were inversely correlated with age. Alagille subjects were stunted and had low body weight and reduced fat and muscle stores. Body mass index z-score was inversely correlated with age despite improved cholestasis. CONCLUSIONS: Children with Alagille syndrome had significant growth deficits as measured by weight, height and upper arm anthropometry at all ages studied. In contrast, subjects with biliary atresia had normal weights and heights. However, body mass index and weight for age z-scores were lower in the older biliary atresia subjects. Few studies of anthropometry in biliary atresia or Alagille syndrome have included the school-aged child. This study addresses this gap in knowledge and provides baseline data for nutritional interventions in these patients.     

Ileus nach nicht endoskopischer PEG-Entfernung

Percutaneous endoscopic gastrostomy (PEG) is used in children for enteral feeding. Complications after removing the PEG tube without an endoscopic procedure are rarely described. We report on a male patient who developed bowel obstruction with perforation and severe peritonitis after removal of the PEG tube without endoscopic assistance; the “bumper” obstructed the ileocecal valve. Endoscopic removal of PEG tubes may avoid similar life-threatening complications.     

Association of serum interleukin-8 levels with the degree of fibrosis in infants with chronic liver disease

OBJECTIVE: Biliary atresia is a neonatal obstructive cholangiopathy characterized by a destructive, obliterative process affecting both the intrahepatic and extrahepatic ducts of the biliary tree that uniquely presents in the first months of life. The consequence of progressive inflammatory and sclerotic reaction is the development of obstructive jaundice. To determine the proinflammatory cytokine profile in children with biliary atresia, we measured circulating levels of interleukin (IL)-1beta, IL-6, tumor necrosis factor-alpha and IL-8. METHODS: Twelve children, five males and seven females, with biliary atresia were studied. In addition, four patients with progressive familial intrahepatic cholestasis and three with Alagille syndrome were also included. Five patients with neonatal hepatitis were studied as controls of a liver disease without portal fibrosis. Serum concentration of total and conjugated bilirubin, gamma-glutamyl transferase and glutamic-pyruvic transaminase were measured by routine methods in all patients at time of sampling for the study. The degree of fibrosis in liver biopsies was scored using the histologic activity index. RESULTS: In our study IL-8 was detectable in 11 of 12 patients with biliary atresia with a median level of 262 pg/ml and a highly statistically significant difference (P < 0.0001) from controls. In patients with progressive familial intrahepatic cholestasis or with Alagille syndrome serum IL-8 levels were similarly elevated. In patients with neonatal hepatitis, IL-8 levels were marginally increased. Serum IL-8 levels were significantly correlated (Rs = 0.725, P < 0.0001) with the histologic activity index. CONCLUSIONS: Although further studies are needed to determine the role of IL-8 in portal inflammation, our results suggest that increased production of IL-8 may be a mechanism leading to the progressive portal inflammation and fibrosis in patients with chronic liver disease.     

Surgery in disabled children: General gastroenterological aspects

Cerebral palsy (CP) is a non-progressive but not unchanging disorder of movement and/or posture, due to an insult to or anomaly of the developing brain. Gastrointestinal surgery can play a role in the treatment of pathologies frequently associated with a condition of neurological impairment such as gastro-oesophageal reflux disease (antireflux procedure), feeding difficulties (percutaneous endoscopic gastrostomy/jejunostomy) and swallowing difficulties (ligation of salivary gland ducts). Gastro-oesophageal reflux occurs in up to 70–75% of children with cerebral palsy. Children with gastro-oesophageal reflux disease (GERD) may present with feeding difficulties, recurrent vomiting and recurrent chest infection associated with poor growth and nutrition, reactive airway disease particularly nocturnal asthma, choking attacks, anaemia, and wheezing. Nutritional deprivation in children with cerebral palsy is the summation of several factors which result in reduced intake. Percutaneous endoscopic gastrostomy (PEG) has radically changed the handling of children with nutritional problems who, before the introduction of this procedure, were force fed parenterally or enterally, by nasogastric tube, conventional surgical gastrostomy or central venous access. In children with CP, PEG is the preferred technique for long-term enteral feeding. Swallowing dysfunction is the main cause of drooling in cerebral palsy, and medical treatment is often inefficient. Surgical treatment involves neurectomy, translocation of the salivary duct, salivary gland resection or salivary duct (parotid and submandibular) ligation.

Conclusion : This review focuses on the role of surgery in managing gastrointestinal aspects in children with CP and, in particular, surgical experience at our department with fundoplication, PEG placement and ligation of salivary ducts.     

Surgery in disabled children: general gastroenterological aspects

Cerebral palsy (CP) is a non-progressive but not unchanging disorder of movement and/or posture, due to an insult to or anomaly of the developing brain. Gastrointestinal surgery can play a role in the treatment of pathologies frequently associated with a condition of neurological impairment such as gastro-oesophageal reflux disease (antireflux procedure), feeding difficulties (percutaneous endoscopic gastrostomy/jejunostomy) and swallowing difficulties (ligation of salivary gland ducts). Gastro-oesophageal reflux occurs in up to 70-75% of children with cerebral palsy. Children with gastro-oesophageal reflux disease (GERD) may present with feeding difficulties, recurrent vomiting and recurrent chest infection associated with poor growth and nutrition, reactive airway disease particularly nocturnal asthma, choking attacks, anaemia, and wheezing. Nutritional deprivation in children with cerebral palsy is the summation of several factors which result in reduced intake. Percutaneous endoscopic gastrostomy (PEG) has radically changed the handling of children with nutritional problems who, before the introduction of this procedure, were force fed parenterally or enterally, by nasogastric tube, conventional surgical gastrostomy or central venous access. In children with CP, PEG is the preferred technique for long-term enteral feeding. Swallowing dysfunction is the main cause of drooling in cerebral palsy, and medical treatment is often inefficient. Surgical treatment involves neurectomy, translocation of the salivary duct, salivary gland resection or salivary duct (parotid and submandibular) ligation. CONCLUSION: This review focuses on the role of surgery in managing gastrointestinal aspects in children with CP and, in particular, surgical experience at our department with fundoplication, PEG placement and ligation of salivary ducts.     

Experience of percutaneous endoscopic gastrostomy in children with Crohn's disease

Accepted 4 November 1996
Enteral nutrition is an important mode of treatment for Crohn''s disease in children. Percutaneous endoscopic gastrostomy has been little used, even though it can facilitate the administration of an unpalatable elemental diet to an anorexic, undernourished patient. Its use is reported in 10 children with Crohn''s disease. The gastrostomy was found to be more acceptable than a nasogastric tube and was associated with only minor complications. As a consequence of improved delivery of enteral nutrition, in the year after the insertion of the gastrostomy there was a reduction in prednisolone dosage in all patients, with six patients being able to stop prednisolone completely. The SD score for height also improved significantly. It is suggested that percutaneous endoscopic gastrostomy is both useful and safe in the management of Crohn''s disease in children, particularly when compliance with an elemental diet is likely to be poor.

     

Biliary atresia: an update on our understanding of the disorder.

Biliary atresia is the leading cause of cholestasis in infants younger than 3 months. It is also the leading indication for liver transplantation in children. This review focuses on recent advances in the etiology, diagnosis, and management of biliary atresia.     

Living donor liver transplantation in Alagille syndrome—Single center experience from south Asia

To analyze the clinical characteristics and the outcomes of living donor liver transplantation in children with Alagille syndrome (AGS). Clinical data of children with AGS who underwent liver transplantation between July 2009 and May 2019 in our unit were retrospectively analyzed. Primary end‐points were patient and graft survival. Ten children with AGS underwent living donor liver transplantation at a median age of 28 months (range, 12‐84 months). Jaundice was the most common initial symptom and was noted after a median duration of 20 days after birth (range, 7‐60 days). Two patients had undergone Kasai porto‐enterostomy for misdiagnosis of biliary atresia. The most common indication for transplantation was severe pruritus with poor quality of life. Explant livers in three children showed cirrhosis with early well‐differentiated hepatocellular carcinoma. We have 100% patient and graft survival at a mean follow‐up of 32 months (range 3‐72 months). The median z‐score for weight and height at liver transplantation was ?2.66 (range: ?6.44 to ?0.9) and ?3.6 (range: ?7.96 to ?0.93) while at follow‐up was ?1.7 (range: ?3.4 to ?0.35) and ?2.1 (range: ?3.9 to ?1.4), respectively. The estimated glomerular filtration rate was normal pretransplant and follow‐up. This is the first series of LDLT for Alagille syndrome in the Indian sub‐continent. We report excellent post‐transplant outcomes in contrast to outcomes reported from Western literature.     

Clinical and pathological characteristics of Alagille syndrome in Chinese children

Background  Alagille syndrome (AS) is regarded as the most common cause of chronic cholestasis in childhood associated with specific phenotypic features in western countries. This study was undertaken to investigate the significance of AS in Chinese children with chronic cholestasis and to describe its clinical and histological features. Methods  From October 2004 to January 2007, 157 children who presented with conjugated jaundice from less than 3 months of age were admitted to a tertiary hospital in Shanghai. Investigations of the heart, spine, eyes and kidneys were conducted in 13 children who experienced prolonged cholestasis beyond 1 year of age after exclusion of biliary atresia and familial progressive intrahepatic cholestasis type 1 or 2. In patients with interlobular bile duct paucity, AS was diagnosed if 3 or more of the following 5 major features were present: cardiac murmur, posterior embryotoxon, butterfly-like vertebrae, renal abnormalities and characteristic faces. In patients without interlobular bile duct paucity or who did not receive liver biopsy, 4 or more features were required for the diagnosis. Results  Of the 13 children, 6 were diagnosed with AS at ages ranging from 1 year and 7 months to 3 years and 11 months. Jaundice was noticed in early infancy and then pruritus developed in all the 6 patients, of whom 5 presented with acholic stool and 4 had been misdiagnosed as having presumed biliary atresia by hepatobiliary scintigraphy or laparoscopic cholangiography. Biochemical examinations demonstrated increased concentration of total bile acid and hyperlipidemia. Interlobular bile duct paucity was demonstrated histologically in 5 patients who received liver biopsy. Vertebral abnormalities, heart murmur, characteristic faces and failure to thrive were found in all the 6 patients. Two patients had evidence of renal involvement. Micropenis, empty scrotum, and gall stone were seen in 1 patient. Conclusion  AS is also an important cause of prolonged cholestasis in Chinese children. It is difficult to differentiate AS from biliary atresia. Liver biopsy and spine X-ray may be helpful in the early detection of AS.     

Contamination of gastrostomy feeding systems in children in a home-based enteral nutrition program

BACKGROUND: There are few data concerning the risk of contamination of enteral feeding systems via gastrostomy in children, and none for conditions that pertain to home-based care. METHODS: To investigate the risk of contamination of enteral feeding systems during the home-based care of 20 children receiving gastrostomy tube feeding, five samples were taken for analysis: two samples before the enteral feeding period (gastrostomy, enteral feeding system) and three after this period (gastrostomy, distal giving set, liquid remained in container). Microorganisms were identified and counted. Different factors were studied to elucidate their role in bacterial colonization: acid suppressive therapy, gastrostomy tube or button, hanging feeding time, rate of enteral feeding, gastric pullulation and retrograde contamination, manipulation error, and use of open or closed enteral feeding systems. RESULTS: Overgrowth was defined as a microorganismal load exceeding 10(4) colony-forming units (cfu)/mL. Overgrowth was present in 85% of gastrostomy samples before enteral nutrition started. Most microorganisms belonged to gastric flora. Some bacteria had an environmental origin or derived from cutaneous flora. Forty-five percent of the lines showed overgrowth at the end of enteral nutrition period, mainly with the same microorganism found in the gastrostomy. Closed enteral bags remained sterile, even if manipulation error occurred. Duration, rate of enteral feeding, and acid suppression treatment were not risk factors for overgrowth. CONCLUSIONS: Retrograde contamination of gastrostomy feeding systems occurs frequently. The preferential use of closed enteral feeding systems is recommended for home-based enteral nutrition programs.     

Intrapulmonary shunting in the biliary atresia/polysplenia syndrome: reversal after liver transplantation

One hundred and seventy three children, including 93 with biliary atresia, received liver grafts at Addenbrooke's Hospital between 1983 and 1993. Of these, only seven developed cyanosis due to intrapulmonary shunting as a complication of their liver disease, and all seven of these had the biliary atresia/polysplenia syndrome. Intrapulmonary shunting was confirmed by a radioisotope scan in four children. Only one child with the syndrome did not have cyanosis when undergoing transplantation. Seven of the eight children are alive 6-54 months after transplantation, with normal pulmonary and hepatic function. Cyanosis recurred in one child who developed chronic rejection with liver failure. In conclusion: (a) there is a strong association between the biliary atresia/polysplenia syndrome and cyanosis due to intrapulmonary shunting; (b) intrapulmonary shunting is fully reversible after successful liver transplantation; and (c) cyanosis, once present, is progressive, and these children should be considered for liver transplantation as soon as it occurs.     

Intrapulmonary shunting in the biliary atresia/polysplenia syndrome: reversal after liver transplantation.

One hundred and seventy three children, including 93 with biliary atresia, received liver grafts at Addenbrooke's Hospital between 1983 and 1993. Of these, only seven developed cyanosis due to intrapulmonary shunting as a complication of their liver disease, and all seven of these had the biliary atresia/polysplenia syndrome. Intrapulmonary shunting was confirmed by a radioisotope scan in four children. Only one child with the syndrome did not have cyanosis when undergoing transplantation. Seven of the eight children are alive 6-54 months after transplantation, with normal pulmonary and hepatic function. Cyanosis recurred in one child who developed chronic rejection with liver failure. In conclusion: (a) there is a strong association between the biliary atresia/polysplenia syndrome and cyanosis due to intrapulmonary shunting; (b) intrapulmonary shunting is fully reversible after successful liver transplantation; and (c) cyanosis, once present, is progressive, and these children should be considered for liver transplantation as soon as it occurs.     

婴儿胆汁淤积症的病因研究进展

婴儿胆汁淤积症是一类多因素引起的以高胆红素血症为临床特点的综合征,病因谱广泛,包括感染、中毒、肝内外胆管发育异常及遗传代谢缺陷等.感染、胆道闭锁患儿占胆汁淤积症患儿半数以上,除了常见病因,citrin缺陷症、Alagille综合征、进行性家族性胆汁淤积症等新病因不断被认识,这些疾病临床症状与胆道闭锁相似,预后及治疗各不...     

Preventing parenteral nutrition liver disease

Parenteral nutrition liver disease (PNLD) develops in 40-60% of infants who require long-term PN for intestinal failure. The clinical spectrum includes hepatic steatosis, cholestasis, cholelithiasis, and hepatic fibrosis. Progression to biliary cirrhosis and the development of portal hypertension and liver failure occurs in a minority who require combined liver and intestinal transplantation.The pathogenesis is multifactorial and is related to prematurity, low birth weight, duration of PN, short bowel syndrome requiring multiple laparotomies and recurrent sepsis. Other important mechanisms include lack of enteral feeding which leads to reduced gut hormone secretion, reduction of bile flow and biliary stasis which leads to the development of cholestasis, biliary sludge and gallstones, which exacerbate hepatic dysfunction, especially in premature neonates with immature hepatic function.The use of lipid emulsions, particularly soy bean emulsions have been associated with hepatic cholestasis in children, although there are little data now to support toxicity from other PN components.Management strategies for the prevention of parenteral nutrition liver disease include consideration of early enteral feeding, a multidisciplinary approach to the management of parenteral nutrition with a specialized nutritional care team and aseptic catheter techniques to reduce sepsis. The use of specialized lipid emulsions such as fish oil emulsions and or SMOF (Soy bean/Medium Chain Triglyceride/Olive Oil/Fish oil) improves established cholestasis and may prevent the onset.Oral administration of ursodeoxycholic acid may improve bile flow and reduce gall bladder stasis, although there is little data to suggest that prophylactic use prevents the onset of PNLD.Survival following either isolated small bowel or combined liver and small bowel transplantation is approximately 50% at 5 years making this an acceptable therapeutic option in children with irreversible liver and intestinal failure.     

Percutaneous endoscopic gastrostomy to set up a long-term enteral feeding route in children: an encouraging result

Percutaneous endoscopic gastrostomy (PEG) is now accepted as the preferred technique to establish long-term enteral feeding. A total of 30 pediatric patients (21 males and 9 females, aged from 5 months to 14 years) underwent PEG in the National Taiwan University Hospital from December 1994 to February 2001. The underlying diseases of the patients receiving PEG were neurological dysfunction ( n=26), gastrointestinal disease ( n=2) and metabolic disorders ( n=2). All patients received intravenous sedation and PEG was performed successfully. Prophylactic antibiotic treatment was given for 1 day. Tube feeding began 48 h after the placement. The Z-score of weight before and 6 months after PEG was -1.54+/-1.74 and -0.98+/-1.46, respectively, which is significant (paired t-test, p<0.0001). Accelerated weight gain was found after PEG. Complications of PEG in our patients included local wound infection ( n=15), gastrocolic fistula ( n=1), pneumoperitoneum ( n=1), dislodgement of tube ( n=6) and tube occlusion ( n=2). Of these patients 12 received button replacement after PEG and the gastrostomy tube was discontinued in 2 patients when they resumed adequate oral intake. Removal of PEG was performed in 2 patients due to complications of gastrocolic fistula and severe dislodgement. Our results encourage the use of PEG as a long-term route for nutritional supply.     

Dumping syndrome: an unusual cause of severe hyperinsulinemic hypoglycemia in neurologically impaired children with gastrostomy

This paper describes severe hyperinsulinemic hypoglycemia during bolus enteral feeding in two neurologically impaired children. Both children were affected by dysphagia with swallowing difficulties; caloric intake was inadequate. For these reasons, percutaneous endoscopic gastrostomy had been positioned during the first months of life. In one patient due to persisting vomiting, after a few months, a gastrojejunal tube (PEG-J) was inserted. Hypoglycemia was revealed by routine blood tests, without evidence of specific symptoms. Continuous subcutaneous glucose monitoring showed wide glucose excursions, ranging from hypoglycemia to hyperglycemia. Extremely high levels of insulin were detected at the time of hypoglycemia. A diagnosis of dumping syndrome (DS) was suspected in both children. In the child with PEG, the tip of the gastrostomy catheter was found to be lying in the bulbus duodeni. Once this had been pulled back, hypoglycemic episodes disappeared. The child with PEG-J needed continuous enteral feeding to reach a normal glucose balance. DS is a relatively common complication in children with gastrostomy, but extremely irregular glucose levels, ranging from hypoglycemia to hyperglycemia, and increased insulin secretion had not been previously demonstrated. The incidence of DS is probably underestimated in children receiving enteral feeding for neurological impairment. In these patients intensive monitoring of blood glucose levels should be performed to calibrate meals. Repeated underestimated hypoglycemic episodes could worsen neurological damage and cause a deterioration in clinical conditions.     

Hepatobiliary scintigraphy and the string test in the evaluation of neonatal cholestasis

We evaluated [99mTc]diisopropylphenyl-carbamoylmethylimidodiacetic acid ([99mTc]DISIDA) cholescintigraphy with measurement of duodenal fluid radioactivity collected by the string test in patients with neonatal cholestasis. Twenty-six infants with prolonged jaundice and acholic stools were studied prospectively. Twelve patients had neonatal hepatitis, 12 biliary atresia, and one each Alagille syndrome and alpha 1-antitrypsin deficiency liver disease. All infants except the biliary atresia patients and four of the neonatal hepatitis patients revealed bowel activity on scan 6 h after tracer administration. At 24 h, three of these latter patients with neonatal hepatitis and two of the patients with biliary atresia revealed bowel activity. String radioactive counts for neonatal hepatitis ranged from 99,574 to 967,205 cpm (374,504 +/- 232,210 cpm; mean +/- SD) and for biliary atresia from 8,342 to 370,346 cpm (117,149 +/- 98,698 cpm; mean +/- SD). While neither test alone was capable of correctly differentiating among all patients, those patients with biliary atresia had either a negative hepatobiliary scan at 24 h or string radioactive count below 197,007 cpm. Disparity between the hepatobiliary scan and the string radioactive counts mandates further diagnostic investigation. These data suggest that simultaneous administration of the string test with hepatobiliary scintigraphy is advantageous in the evaluation of infants with cholestatic jaundice.     

Limitations of percutaneous endoscopic gastrostomy in facilitating enteral nutrition in children: review of the shortcomings of a new technique

Percutaneous endoscopic gastrostomy (PEG) insertion became the preferred technique for facilitating enteral nutrition in children unable to take adequate caloric intake orally once its advantages over the standard Stamm gastrostomy became apparent. It has taken longer for some of its limitations and shortcomings to be recognized. Problems encountered during PEG insertion include: inability to enter the stomach with the trocar, oesophageal laceration, colonic perforation, gastro-colo-cutaneous fistula, peritonitis, subcutaneous emphysema, external migration of inner flange, wound infection, peristomal excoriation, symptomatic gastro-oesophageal reflux requiring later fundoplication, intestinal obstruction and haemorrhage. Many of these complications can be avoided by attention to technique.     

Arthrogryposis, renal tubular dysfunction, cholestasis (ARC) syndrome: case report and review of the literature

The ARC-syndrome is a rare disease with the obligatory symptoms arthrogryposis, renal tubular dysfunction and cholestasis. Optional further symptoms like ichthyosis, diarrhea, central nervous system defects and recurrent infections have been reported. The ARC-syndrome was first reported by Lutz-Richner and Landolt in 1973. The pathophysiology is still unknown, an autosomal recessive inheritance is postulated. Patients rarely exceed an age of six month. We report a boy of consanguineous Turkish parents who suffered from congenital deformities of the lower extremities, a metabolic acidosis and failure to thrive. In the sequel he developed a renal Fanconi syndrome and cholestasis. Histology of liver and muscle biopsy specimen showed the typical findings of the disease with giant cell hepatitis and neurogenous muscle atrophy. His condition could be stabilized and he increased in weight by substituting fluid, electrolytes, buffer and parenteral nutrition. Total enteral nutrition of the 280 ml/kg/d he required failed even by nasogastric tube and percutaneous endoscopic gastrostomy. Additional fluid substitution by central venous catheter remained necessary. At the age of 7 month he died.