Chronic Intestinal Pseudo-obstruction Syndrome Research Articles

Chronic intestinal pseudo-obstruction syndrome in pediatric patients: O. Goulet, A. Jobert-Giraud, J.-L. Michel, et al. Eur J Pediatr Surg 9:83–90, (April), 1999

Chronic intestinal pseudo-obstruction syndrome in pediatric patients: O. Goulet, A. Jobert-Giraud, J.-L. Michel, et al. Eur J Pediatr Surg 9:83–90, (April), 1999

Chronic intestinal pseudo-obstruction: treatment and long term follow up of 44 patients

AIMSTo document the long term course of chronic idiopathic intestinal pseudo-obstruction syndrome (CIIPS) in children with defined enteric neuromuscular disease, and the place and type of surgery used in their...

Chronic intestinal pseudoobstruction syndrome: clinical analysis, outcome, and prognosis in 105 children. French-Speaking Group of Pediatric Gastroenterology.

Our aim was to collect a large number of cases to characterize clinical presentation, outcome, and prognosis of chronic intestinal pseusoobstruction in children. We conducted a retrospective multicenter study that included children treated for chronic intestinal pseusoobstruction defined as recurrent episodes of intestinal obstruction with no mechanical obstruction, excluding Hirschsprung's disease. In all, 105 children, 57 boys and 48 girls, were studied, including five familial forms. Prenatal diagnosis was made in 18 patients. Eighty patients were less than 12 months old at onset; the disease began at birth for 37 patients. The most frequent signs were abdominal distension, vomiting, and constipation. Megacystis was noted in myopathies (7 cases), neuropathies (10 cases) and unclassified forms (13 cases). For all but three cases (two patients with CMV infection, one with Munchhausen-by-proxy syndrome), the associated diseases and disorders could not account for chronic intestinal pseusoobstruction as a secondary disorder. At least one full-thickness biopsy from the digestive tract was studied for 99 patients. The diagnosis recorded was visceral neuropathy in 58 cases, visceral myopathy in 17 cases, and uncertain or normal biopsy results in 24 cases. Seventy-eight children were fed intravenously, and only 18 were able to be fed orally throughout their illness. Seventy-one patients underwent surgery during their illness, and 217 surgical procedures, a mean of 3 per patient, were performed. Ostomy was the most performed procedure. Follow-up continued in 89 patients for 3 months to 16 years (mean 85 months). Forty-two patients were still fed by parenteral (39 patients) or enteral nutrition (3 patients) at the time of the study. Eleven patients died between the age of 1 month and 14 years 7 months.

Intestinal failure in children

Intestinal failure (IF) is a condition in which severe intestinal malabsorption requires parenteral nutrition (PN). Causes of protracted intestinal failure include short bowel syndrome (SBS), congenital diseases of enterocyte development (CDED), and severe motility disorders (total or subtotal aganglionosis or chronic intestinal pseudo-obstruction syndrome). IF can result in “nutritional failure,” defined as the incapacity to continue to feed a child by using PN. Today, intestinal failure-associated liver disease (IFALD) is the most common cause of nutritional failure, but catheter-related sepsis and extensive vascular thrombosis may also jeopardize the use of long-term PN. For a child with nutritional failure, intestinal transplantation (ITx), often in the form of a composite visceral graft, offers the only option for long-term survival. The management of IF requires a multidisciplinary approach. There have been a number of recent advances in both medical and surgical treatments of IF. In particular, new intestinal lengthening techniques and the use of parenteral nutrition formula rich in fish oil have both resulted in decreased rates of severe complications of IF and its treatment. In addition, improved awareness of the risks and benefits of ITx have resulted in better patient selection, and ultimately in improved patient survival, leading to restrict the indication to ITx only to patients with nutritional failure with no other chance to survive.

Small-intestinal transplantation

The management of patients with intestinal failure has benefited from progress in parenteral nutrition (PN), especially home-based PN. Intestinal transplantation is therefore possible and is now, in some conditions, the logical therapeutic option. Since 1985, more than 180 small-bowel grafts have been done, involving the isolated small bowel with or without the colon (38%), the liver-small bowel (46%) or several organs (16%). Two-thirds of recipients were under 20 years of age, and indications were short-bowel syndrome (64%), severe intractable diarrhoea (13%), abdominal cancer (13%) or chronic intestinal pseudo-obstruction syndrome (8%). Of the patients, 51% survived > 2 years after the graft. Patient and graft survival depends on the type of immunosuppression, i.e. cyclosporine or FK506. The results must be interpreted carefully as they represent the first experience in numerous centres using different immunosuppressive protocols, without any randomization. The results from the largest of these centres reflect the current situation more closely. Functional grafts lead to gastrointestinal autonomy (weaning of PN) while maintaining satisfactory nutritional status and normal growth in childhood. Intestinal transplantation is theoretically indicated for all patients permanently or dependent for a long time on PN. However, as PN is generally well tolerated, even for long periods, each indication for transplantation must be carefully weighed up in terms of the iatrogenic risk and quality of life. When PN has reached its limits, especially in those associated with vascular, infectious, hepatic or metabolic complications, intestinal transplantation must be undertaken. Transplantation of the small bowel alone remains the first option, as combined liver-small bowel grafting is only indicated in the case of life-threatening progressive cirrhogenic liver disease.

Intestinal transplantation in pediatric patients: The European experience

Since 1985, more than 180 small bowel grafts have been performed, essentially in the United States, France, and Canada. Approximately two-thirds of recipients were under 20 years of age (< 5 years, 46% and 5 to 20 years, 18%). The transplants involved the isolated small bowel with or without the colon (38%), the liver and small bowel (46%), or several organs (16%), including the stomach, pancreas, liver, and small bowel. The main indications were short-bowel syndrome (64%), severe refractory diarrhea (13%), abdominal cancer (13%), or chronic intestinal pseudo-obstruction syndrome (8%). These data from the international register show that about half of the 180 reported small bowel transplantations, isolated (SBTx) or combined with the liver (SBLTx), involved children under 16 years of age. Since 1987, 20 small bowel transplantations, 14 SBTx and 6 SBLTx, have been performed in 18 children in Europe, 13 under cyclosporine (CyA) and 7 under FK 506 (Tables 1 and 2). The longest survival is 7 3 4 years for a still living 8 1 4 year-old girl who received an isolated small bowel graft from an anencephalic neonate. She is living at home, totally orally fed, growing normally, and treated with CyA (Neoral) and low-dose steroids (0.1 mg/kg/d). Three other patients are currently alive in France, 4, 21, and 24 months after SBLTx for extreme short bowel syndrome, microvillous atrophy, and epithelial dysplasia associated with end-stage liver cirrhosis. All have discontinued total parenteral nutrition (TPN) and are orally fed. They receive FK 506 between 0.2 and 0.4 mg/kg/d to achieve whole blood levels around 10 ng/mL, and low doses of steroids.

Chronic intestinal pseudo-obstruction syndrome in two patients with pancreatic somatostatioma

Chronic intestinal pseudo-obstruction syndrome in two patients with pancreatic somatostatioma

Manometric evaluation of gastrointestinal motility in children with chronic intestinal pseudo-obstruction syndrome

Various causes of abnormal gastrointestinal motility may account for the symptoms of chronic intestinal pseudoobstruction syndrome (CIIPS). Fasting gastrointestinal motility in humans is characterized by regular cyclic activity of the migrating motor complex (MMC), which may be used as an objective indicator of intestinal motility. The present study investigated MMC in five children with CIIPS (2 neurogenic and 3 nonneurogenic cases) using a perfusedcatheter system. The findings were compared with those for children with idiopathic constipation (9) and Hirschsprung's disease (4). The gastrointestinal MMC was found to be abnormal with CIIPS, whereas it remained normal with idiopathic constipation and Hirschsprung's disease. The four CIIPS patients without any gastrointestinal MMC activity required total pareteral nutrition. There was significant improvement of intestinal motility over time in a patient with immaturity of ganglia, and there was gradual deterioration in a patient with hypoganglionosis. In conclusion, gastrointestinal motility studies provide objective diagnostic information in patients with intestinal dysmotility and can be used as an indicator of intestinal function during the treatment of CIIPS.

Neonatal-onset chronic intestinal pseudo-obstruction syndrome.

Between January 1985 and January 1990, six cases of neonatal-onset chronic intestinal pseudo-obstruction syndrome (CIPS) were identified. Failure to gain weight in six cases, abdominal distention in five, and vomiting in five were the most common presenting symptoms. The contrast studies of the gastrointestinal tract demonstrated delayed transit time in 6/6, jejunal or ileal dilatation in 1/6, megaduodenum in 1/6, dilatation of the colon with barium retention in 4/6, and microcolon in 1/6. Urinary tract involvement was noted in three patients. Laparotomy, performed in three patients, revealed no mechanical obstruction. Except for hypoganglionosis in Patient 4, no recognizable neuropathy or myopathy was noted histopathologically. Four patients expired within 2 months after discharge. We conclude that CIPS with neonatal onset should be suspected when infants have urinary retention and abdominal distention or constipation beginning at birth or soon after. The prognosis of CIPS presenting in the newborn period appears worse than that presenting in childhood or adulthood.

230 Patient years of experience with home long-term parenteral nutrition in childhood: Natural history and life of central venous catheters

The records of 27 pediatric patients who required parenteral nutrition (PN) for 5 to 14.5 years (mean ± SD, 8.5 ± 3.8) were analyzed to determine the frequency of complications with their central venous catheters (CVC). This represents a 230 patient-year experience. Patients with short bowel syndrome and chronic intestinal pseudoobstruction syndrome (CIPS) accounted for all but two of the patients. Unsuccessful medical management of the exit site or CVC infection was responsible for removal of 62% of the 123 CVCs. Ninety-five episodes of line infection occurred in 24 patients. Fifty (52%) were successfully treated without catheter removal. The organisms responsible for catheter removal were fungal (14), mycobacterium species (5), gram-positive cocci (22), or gramnegative bacilli (19). The CVCs were infected an average of once every 884 days. The life of the second CVC (23.5 ± 17.9 months) was significantly longer than that of the first ( P < .05). Clotting of the CVC with unsuccessful lysis of the clot was responsible for removal of 24%, and breakage or unsuccessful repair was responsible for 14%. In no patient were all possible venous sites for CVC placement exhausted. Patients with CIPS had substantially fewer catheter complications ( P < .05) than did those with short bowel syndrome. In conclusion, CVCs can “survive” without major complications for more than a decade. Numerous factors contribute to the increased rate of CVC survival over time, including improvement in PN self-care with greater experience, improvement in teaching, regular follow-up of patients, better management of infection, and better ability to treat CVC thrombosis or breakage.

Cisapride. An updated review of its pharmacology and therapeutic efficacy as a prokinetic agent in gastrointestinal motility disorders.

Cisapride is an orally administered prokinetic agent which facilitates or restores motility throughout the length of the gastrointestinal tract. It is a substituted piperidinyl benzamide, chemically related to metoclopramide, but unlike metoclopramide, cisapride is largely devoid of central depressant or antidopaminergic effects. In placebo-controlled trials, cisapride improved healing rates and symptoms in both adults and children with reflux oesophagitis. Maintenance therapy with cisapride at half the healing dose is effective in reducing the incidence of relapse. Symptoms are also alleviated in patients with functional dyspepsia, and gastric emptying and symptoms are improved in most patients with gastroparesis, an effect which is sustained during long term administration. However, the efficacy of cisapride in end-stage gastroparesis remains less clear. Cisapride increases stool frequency in patients with chronic constipation, and limited data suggest that the drug may also be beneficial in treating chronic intestinal pseudo-obstruction and irritable bowel syndrome. Cisapride demonstrated efficacy comparable with or superior to that of metoclopramide, and was at least as effective as cimetidine and ranitidine in patients with reflux disease. In patients with functional dyspepsia, cisapride has shown at least equal efficacy to domperidone, metoclopramide and ranitidine, and superior efficacy to cimetidine in the small comparative trials conducted to date. Adverse effects in patients receiving cisapride are generally transient and mild, with abdominal cramping, borborygmi, diarrhoea or loose stools most frequently reported. Central nervous system adverse effects are rare. Thus, with its favourable tolerability profile and demonstrated efficacy in a variety of gastrointestinal motility disorders, the position of cisapride as a valuable agent in the management of patients with gastrointestinal motility disorders is strengthening. However, larger well-controlled comparative trials of the drug with other agents are necessary before the relative position of cisapride in therapy can be categorically defined.

Clinical, laboratory and prognostic features of congenital large intestinal motor dysfunction (pseudo-Hirschsprung's disease).

One hundred and forty-eight cases of congenital large intestinal motor dysfunction (pseudo-Hirschsprung's disease) were reported by members of the Japanese Society of Pediatric Surgeons during the past 20 years. The disorder was defined as a congenital, non-mechanical obstruction of the intestine with the presence of intramural ganglia in the terminal rectum. Intramural ganglia were abnormal in 77 cases, normal in 42, and could not be determined in 29. Of those with abnormal intramural ganglia, 54 had immature ganglia or hypoganglionosis (oligoganglionosis), 15 had neuronal intestinal dysplasia, and eight had a segmental anomaly. Of those with a normal myenteric plexus, 22 had chronic and twelve had suspected idiopathic intestinal pseudo-obstruction syndrome; eight had megacystis-microcolon-intestinal hypoperistalsis syndrome. While cases with both hypoganglionosis and normal intramural ganglia had normal acetylcholine esterase activity, a significantly greater number of patients with hypoganglionosis lacked normal rectoanal reflexes. Patients with hypoganglionosis, chronic idiopathic intestinal pseudo-obstruction syndrome, and megalocystis-microcolon-intestinal hypoperistalsis syndrome had poor prognoses with an overall mortality of 36.9%. These findings indicate that congenital large intestinal motor dysfunction remains a serious disease of childhood.

Biliary tract complications in patients with hypoganglionosis and chronic idiopathic intestinal pseudoobstruction syndrome

The biliary tract system was studied in two patients with hypoganglionosis and chronic idiopathic intestinal pseudoobstruction syndrome (CIIPS) to evaluate manifestations of these diseases, especially as for underlying motility disorder. In a 3-year-old boy with hypoganglionosis, cholelithiasis was diagnosed and the gallbladder specimen showed a markedly hypoplastic neural plexus on histopathology as was found in his total alimentary tract. In a 2-year-old girl with CIIPS, echo-guided gallbladder wall motility testing demonstrated an impaired response to a ceruletide diethylamine stimulation. These clinical experiences in two patients suggest that these pathological entities may be frequently associated with biliary tract complications not only due to a consequence of total parenteral nutrition, but also due to a possible intrinsic involvement of biliary tract dysmotility.

Chronic intestinal pseudoobstruction syndrome

Chronic intestinal pseudoobstruction syndrome

Neuroendocrine design of the gut

The enteric nervous system (ENS) can be thought of as the third component of the autonomic nervous system. It is a vast network of neurons widely dispersed throughout the gut. The ENS is a dominant regulator of gut function through the action of peptide and non-peptide neurotransmitters. The most intensively studied roles of the ENS have been the regulation of secretory processes, such as gastric acid secretion, and motility. It is clear, however, that the ENS plays a broader role in the regulation of other gut functions, including mucosal defense, the gut immune response, and sphincter function. Alterations in the regulation of gut function by the ENS are likely or suspected in a number of conditions, including achalasia, Hirschsprung's disease, inflammatory bowel disease, Chagas' disease, chronic intestinal pseudoobstruction, biliary dyskinesia, tachygastria, and irritable bowel syndrome. Improved knowledge of the pathophysiology of these troublesome conditions makes effective therapy more likely in the future.

Total Parenteral Nutrition‐Induced Steatosis: Reversal by Parenteral Lipid Infusion

Prolonged use of total parenteral nutrition (TPN) may be associated with hepatic complications, primarily steatosis and cholestasis. A case is reported of an 18-year-old woman with chronic idiopathic intestinal pseudo-obstruction syndrome who was on prolonged home parenteral nutrition without lipid supplementation and developed steatosis. This finding was reversed by addition of lipid emulsion, at a dose of 0.5 g/kg/day, to the parenteral nutrition solution. The lack of lipid supplementation as a possible cause of steatosis, as well as other mechanisms of liver steatosis associated with TPN, are discussed.

Visceral Neuropathies Responsible for Chronic Intestinal Pseudo-Obstruction Syndrome in Pediatric Practice

Twenty-six children aged from 1 day to 15 years (18 boys and 8 girls) with visceral neuropathies are analyzed. Clinical symptomatology is dominated by abdominal distension, attacks of occlusion, abdominal pain, and malnutrition. Intestine bacterial overgrowth is frequent. From aspiration biopsies, surgical rectal biopsies, and, in some cases, ileal or ileocolic biopsies, histopathological studies revealed two patterns. One group had abnormalities of the myenteric plexus identified by conventional light microscopic studies, with two patterns: [myenteric plexus hyperplasia (9 patients), characterized by large ganglionic nodes, penetration into the mucosal zone, and altered argyrophilic neurons]. Clinically this pattern was observed in four patients with multiple endocrine neoplasia syndrome with risk of medullary thyroid carcinoma. The second pattern observed was characterized by glial cell hyperplasia (15 patients). Ganglion cells are present but are small and sparse, often infiltrated by collagen tissue; Schwann nerve fibers are hypertrophic. Eleven patients presented with neonatal intestinal obstruction. The second group is characterized by normal conventional light microscopic examination, but silver stains revealed important abnormalities of argyrophobic cells (one case) or argyrophilic cells (one case). In the two groups, most of the patients needed intestinal derivation and prolonged nutritional support with total parenteral nutrition.

Primary intestinal myopathy, a cause of chronic idiopathic intestinal pseudoobstruction syndrome (CIPS): clinicopathological studies of seven cases in children.

Clinicopathological data on seven instances of primary intestinal myopathy in children are reported. The ages of the patients ranged from eleven months to thirteen years. A persistent intestinal obstruction was the main and constant clinical feature. An ineffective intestinal propulsion was documented on manometric studies. Various urological abnormalities were present in three cases. One patient died and six survive but are dependent on enteral and parenteral nutrition. The morphological findings consisted of degenerative changes involving the muscular layers of the intestinal wall. These changes varied from cytoplasmic vacuolation to definite atrophy and disappearance of the muscular fibers. An extensive interstitial fibrosis underlined these atrophic changes in the late stages of the disease. A familial history was identified in three cases, one consistent with an autosomal dominant transmission.

Chronic Intestinal Pseudo-obstruction Syndrome in Pediatrics

A total of 87 cases of chronic intestinal pseudo-obstruction were identified as fitting the criteria for the syndrome. Five families, three with autosomal dominant inheritance, accounted for 15 cases. The remainder were single cases in families, indicating either autosomal recessive inheritance, spontaneous mutation, or acquired disease. Of the 87 patients, 47 were male; 19 patients were symptomatic at birth and 37 (43%) within the first month of life; 64% were diagnosed by the first year of age and the remainder were diagnosed by 18 years of age. Patients currently range in age from 3 months to 24 years. Abdominal distension in 70, vomiting in 50, and constipation in 50 of the 87 cases were the three commonest presenting symptoms. Diarrhea and failure to thrive were present in 20 cases. Urinary tract infection and failure to void were present in 10 cases. Diagnosis was established by clinical presentation, radiographic studies, and at exploratory laparotomy. Esophageal motility was abnormal in 14 patients. Anal manometric studies were done in 16 and showed normal recto-anal inhibitory reflex in all but one. Only 34 patients had biopsy studies as part of their evaluation and, of these, only 12 were full-thickness. Abnormal plexuses were found in eight and degeneration of smooth muscles in four. There was little or no benefit from the use of any medication to promote motility. One patient with intractable pseudo-obstruction benefited from a subtotal enterectomy. Of those patients not lost to follow-up, 31.4% died; 43% of these deaths occurred within the first 6 months from complications of total parenteral nutrition. One infant had a complete spontaneous remission by 1 year of age, with normalization of radiographic findings; another had partial remission by 6 months of age.

僧帽弁逸脱症候群を伴った慢性特発性偽性腸閉塞症の１例

僧帽弁逸脱症候群を伴った慢性特発性偽性腸閉塞症の１例