Treatments for pediatric achalasia: Heller myotomy or pneumatic dilatation?

AIM: The treatment of achalasia consists of reducing distal esophageal obstruction by either Heller myotomy surgery or endoscopic pneumatic dilatation. The aim of the present study was to evaluate the short- and middle-term results of these procedures in children. METHODOLOGY: For technical reasons, children under six years old (n=8) were treated by surgery only, whereas patients over six years old (n=14) were treated by either Heller myotomy or pneumatic dilatation. RESULTS: Of the children aged under six years, 75% were symptom-free at six months and 83% at 24 months of follow-up. Of the patients aged over six years, complete remission was achieved by Heller myotomy in 44.5% vs. 55.5% by pneumatic dilatation after six months, and in 40% vs. 65%, respectively, after 24 months. Both pneumatic dilatation and Heller myotomy showed significant rates of failure. CONCLUSION: These results suggest that pneumatic dilatation may be considered a primary treatment in children over six years old. Also, where necessary, Heller myotomy and pneumatic dilatation may be used as complementary treatments.

[Psychosomatic approach to encopresis]. / Abord psychosomatique de l'encoprésie.

Encopresis most often results from functional constipation and a behaviour disorder characterised by retention of faeces. Rarely it is a passive or active expulsion of normal faeces. It indicates a failure in the education of sphincter control, often with a preferential development of autoerotic versus relational investments. A depressive component is frequent. We propose a bidisciplinary approach with a somatic and psychological evaluation of the encopretic child from the first visit. The physical examination assesses constipation and stercoral stasis. Associated psychopathological symptoms or a pathogenic psychosocial situation must be sought. The therapeutic means must be directed towards the different etiologic features. Explanations of the physiopathology of the symptom and discussion with the child and the parents on the origin of the dysfunction must be accomplished first. A medical treatment of the constipation is generally indicated. Psychotherapy is initiated according to the background and associated psychopathological symptoms.

[Sleeping position, prevention of sudden death syndrome and gastroesophageal reflux]. / Position de sommeil, prévention de la mort subite du nourrisson et reflux gastrooesophagien.

Based on results of epidemiological studies, dorsal or lateral sleeping positions are now recommanded in the prevention of sudden infant death syndrome (SIDS). This raises an ethical question about the attitude towards the ventral positioning therapy for gastroesophageal reflux (GOR). The consensus conference considers that the ventral position should only be recommanded in GOR when the benefit appears to outweigh the risk of SIDS that it induces. The conference proposes that for infants with simple uncomplicated reflux, sleeping in the prone position should not be introduced in the first line treatment. Prone positioning should be restricted to complicated cases resistant to dietary and medical measures.

Manometrical evaluation in visceral neuropathies in children.

Manometrical recordings were made at three levels of the digestive tract in 20 children with chronic intestinal pseudoobstruction (CIPO) defined clinically and histopathologically by deep biopsies showing a neuropathic process. Duodenal manometry showed severe abnormalities with hypomotility in all cases and absence of migrating motor complex in 13 of 20 cases. There was no relation between the histopathologic type and the motility pattern, but the most severe abnormalities were seen in the patients with extensive involvement of the digestive tract and the most severe clinical course. Esophageal manometry was abnormal in 18 of 19 patients, with altered peristalsis consisting of simultaneous, short-lasting, or low-amplitude waves. Rectoanal manometry showed the presence of the rectosphincteric inhibitory reflex in all patients. In conclusion, there is a high frequency of small bowel manometrical abnormalities in CIPO which seem to correlate with the extent of the pathological process and the prognosis of the disease. Esophageal manometry is useful for defining the extent of dysmotility and confirming the diagnosis of CIPO in some cases.

Duodenal and esophageal manometry in total colonic aganglionosis.

Eleven children with total colonic aganglionosis were studied by esophageal and duodenal manometry. They were separated into two groups: group 1, with ileal involvement, and group 2, without ileal involvement. The results of the digestive motility studies were compared with the extent of ileal involvement of the aganglionosis and with the amount of artificial nutritional support required. All children showed abnormalities on both esophageal and duodenal manometry, suggesting complete digestive tract motility involvement. Recent advances in understanding Hirschsprung's disease and manometrial abnormalities suggest a primary motility disorder in children with total colonic aganglionosis.

Duodenal manometry in postobstructive enteropathy in infants with a transient enterostomy.

Intestinal motility was studied in 11 children with a transient enterostomy secondary to a neonatal organic small intestine obstruction (5 total colon Hirschsprung's disease, 2 necrotizing enterocolitis, 1 intussusception, 3 ileal atresia). Eight children presented with a postobstructive enteropathy (severe grade I [5], moderate grade II [3]) and three were considered as controls (grade III). They were assigned to one of the three groups on the basis of the duration of parenteral nutrition and constant rate enteral nutrition needed and the oral feeding tolerance. Barium small intestine transit showed no persistent partial obstruction or peritoneal adhesions. The abnormal inert marker transit times were statistically correlated with the clinical groups as well as duodenal manometric abnormalities. Manometric recordings were characterised by the absence (grade I) or abnormal phase III (grade II) of the migrating motor complex and decreased motility index (grades I and II). This study confirms that this enteropathy is due to a chronic alteration in motility induced by prenatal or postnatal obstructions.

A proposition for the diagnosis and treatment of gastro-oesophageal reflux disease in children: a report from a working group on gastro-oesophageal reflux disease. Working Group of the European Society of Paediatric Gastro-enterology and Nutrition (ESPGAN).

In this paper, a Working Group on Gastro-Oesophageal Reflux discusses recommendations for the first line diagnostic and therapeutic approach of gastro-oesophageal reflux disease in infants and children. All members of the Working Group agreed that infants with uncomplicated gastro-oesophageal reflux can be safely treated before performing (expensive and often unnecessary) complementary investigations. However, the latter are mandatory if symptoms persist despite appropriate treatment. Oesophageal pH monitoring of long duration (18-24 h) is recommended as the investigation technique of choice in infants and children with atypical presentations of gastro-oesophageal reflux. Upper gastro-intestinal endoscopy in a specialised centre is the technique of choice in infants and children presenting with symptoms suggestive of peptic oesophagitis. Prokinetics, still a relatively new drug family, have already obtained a definitive place in the treatment of gastro-oesophageal reflux disease in infants and children, especially if "non-drug" treatment (positional therapy, dietary recommendations, etc.) was unsuccessful. It was the aim of the Working Group to help the paediatrician with this consensus statement and guide-lines to establish a standardised management of gastro-oesophageal reflux disease in infants and children.

The effect of artificial feeding on cholestasis, gallbladder sludge and lithiasis in infants: correlation with plasma cholecystokinin levels.

The occurrence of hepatic cholestatis (judged by fasting serum bile acid levels), gallbladder sludge formation and lithiasis (ultrasonography) and their correlation with plasma cholecystokinin (CCK) levels was studied in a group of children on continuous total parenteral nutrition (TPN) (n = 95), and later in 40 of these children on cyclic TPN (cTPN). After resumption of oral feeding, 75 were studied on partial oral feeding (2-4 meals) and 40 on constant rate enteral nutrition (CREN) then 45 on total oral feeding (4-6 meals). Gallbladder sludge occurred in 23% of the children on TPN for 1 month and 32% of those on cTPN for 3 months. On CREN, the sludge rate was unchanged, but dropped significantly (17%) on partial oral feeding, and disappeared in children on total oral feeding. Serum bile acids were abnormal in 80% of children on TPN or cTPN and diminished significantly on total oral feeding only. Plasma CCK levels on TPN, cTPN and CREN were identical to fasting levels of children on total oral feeding. Plasma CCK levels increased significantly 1 h post-prandially during both partial (p < 0.02) and total oral feeding (p < 0.001). There was a significant negative correlation between the gallbladder sludge rate and CCK levels for all methods of feeding. This study demonstrates the frequent occurrence of hepatic cholestasis in infants, and the much lower frequency of gallbladder sludge in children compared to adults on TPN. Plasma CCK levels obtained during the different methods of feeding could explain the reduction and eventual disappearance of sludge following stimulation of CCK secretion by discontinuous feeding.

Visceral neuropathies responsible for chronic intestinal pseudo-obstruction syndrome in pediatric practice: analysis of 26 cases.

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.

Microscopic colitis: a new cause of chronic diarrhea in children?

From a retrospective study on children who underwent colonoscopy or rectosigmoidoscopy with multiple level biopsies, we selected five patients whose rectocolonic endoscopic aspect was normal and contrasting with the presence of a microscopic colitis on biopsies. These five children had chronic diarrhea (mean duration of 14 months), associated with vomiting (three cases), abdominal pain (two cases), anorexia (two cases), abdominal distension (two cases), and weight loss (four cases). Symptomatic treatment was used in all children: loperamide (one case), trimebutine (three cases), and aluminium and magnesium silicate (two cases). One child received sulfasalazine for 2 months. After 1 year, all patients had normal stools. Rectosigmoidoscopy was performed in four patients and was normal. Biopsies obtained in three cases were normal in two cases and showed a persistent microscopic colitis in one case. Microscopic colitis may be a distinct cause of chronic diarrhea in children.

[Neurocristopathy. The association of Hirschsprung's disease-ganglioneuroma with autonomic nervous system dysfunction in 2 children]. / Neurocristopathie. Association "Maladie de Hirschsprung-ganglioneuromes et dysfonctionnement du système nerveux autonome" chez 2 enfants.

Two children with the coexistence of long segment Hirschsprung's disease, ganglioneuroblastoma with Ondine's curse in one case and autonomic nervous system impairment in the other are presented. All lesions may be related to aberrations of neural crest cell growth, their migration, or differentiation. A genetic etiology, and the link between Hirschsprung's disease and multiple endocrine adenomatosis is discussed. The combination of Hirschsprung's disease and a neurocristopathy strongly suggests that exploration of the autonomic nervous system and APUD system be carried out.

[Upper jejunal atresia: treatment using jejunal resection, duodenal modeling and duodeno-jejunal or ileal anastomosis. Results in 13 newborn infants]. / Atrésies jéjunales hautes: traitement par résection jéjunale, modelage duodénal et anastomose duodéno-jéjunale ou iléale. Résultats chez 13 nouveau-nés.

13 high jejunal atresias, including 6 type IIIb apple peel small bowel deformities, have been treated by a one stage surgical procedure, including proximal jejunal resection, tapering duodenoplasty and end-to-end duodeno-jejunal or duodeno-ileal anastomosis. All 13 infants survived. There were no anastomotic leak or malfunction. 7 jejunal atresias with normal distal small bowel were fed a mean of 9 days post-operatively (range 4 to 12 days). Intra-venous fluids were discontinued at a mean of 18 days post-operatively (range 13 to 24 days). Patients, all premature babies, were discharged on the mean 30th post-operative day (range 24 to 40). 5 jejunal atresias with apple peel deformity were fed with an average post-operative delay of 21 days (range 12 to 24). All intra-venous fluids were discontinued at a mean of 48 days post-operatively (range 35 to 90). Average hospital stay was 78 days (45 to 120). In one case, an ileal diaphragm, unnoticed during the first operation was subsequently resected at age 45 days and an iterative tapering duodenoplasty was performed at the age of 8 months. Recovery was then uncomplicated and the child was discharged on a normal oral feeding at the age of 10 months. Functional obstruction of the intestine at the site of anastomosis often occurs in high jejunal atresias. 70% of mortality resulted from operative complications in atresias with apple peel deformity. Intestinal distension is the cause of anastomotic malfunction. After jejunal resection, the duodenum remains dilated and anastomosis cannot function properly.(ABSTRACT TRUNCATED AT 250 WORDS)

[Multiple intestinal atresias. Report of 2 cases]. / Atrésies intestinales multiples. Rapport de deux observations.

We report two new cases of multiple gastrointestinal atresias as described by Guttman and al in 1973. The clinical presentation is characteristic: the newborn presents himself with a neonatal occlusion. Plain abdominal film shows gas in a distended stomach with intraluminal calcifications. At laparotomy in both cases a diaphragm was present at the pyloric region followed by innumerable diaphragms only a few centimeters apart until the rectum in the first case, and the right colon in the second one. Reconstruction of the intestinal lumen was impossible and the babies died due to sepsis. Pathologic finding in both cases were those of numerous intraluminal diaphragm associated in the first cases with types II atresias. There was no normal epithelium from the stomach to the rectum. This strongly supports the hypothesis of a malformation of the gastrointestinal tract occurring during early fetal life. The possibility of an autosomic recessive transmission of the process was outlined by Guttman.

Trimebutine-induced phase III-like activity in infants with intestinal motility disorders.

Duodenal manometric recordings were performed in five male children (mean age 11.7 +/- 6.8 months) suffering from severe digestive pathology with clinical findings of dysmotility; they required total parenteral nutrition: one case of enteropathy following intestinal resection for congenital small bowel atresia, and four cases of intestinal pseudoobstruction. The basal 3-h fasting recordings showed complete disorganization of interdigestive activity characterized by an absence of migrating motor complexes and a marked basal hypomotility with motor indices lower than in control subjects. Intravenous trimebutine (3 mg/kg) produced a phase III-like activity 88 +/- 121 s after administration in four cases. The activity lasted 236 +/- 105 s and had a mean frequency of 11.75 +/- 0.86 waves/min. It was propagated aborally in the two patients having two duodenal recording sites. Trimebutine-induced phase III activity was followed by signs of peristalsis in two patients.

Enterocytic differentiation and glucose utilization in the human colon tumor cell line Caco-2: modulation by forskolin.

The human colon cancer line Caco-2 exhibits after confluency a concomitant increase of glycogen accumulation and an enterocytic differentiation. The purpose of this work was to investigate whether forskolin (FK), an activator of adenylate cyclase, would induce a permanent glycogenolysis and, if so, whether it would result in modifications of the differentiation pattern of the cells. FK activates adenylate cyclase in Caco-2 cells with an ED50 of 7 X 10(-6)M. Three different treatment protocols with FK (10(-5)M) were applied: 1) the cells were treated during all the time in culture (20 days); 2) the treatment was started after confluency; 3) the treatment was interrupted after confluency. The presence of FK results in a permanent stimulation of cAMP accumulation (10 to 20 fold the basal values) and in a permanently reduced glycogen content (30 or 50% of the control values). The rates of glucose consumption are increased three and five fold in protocols 1 and 3 respectively. These metabolic changes are associated with morphological changes (tightening of the intercellular spaces and shortening of the brush border microvilli) and with a dual inhibition of the activities of brush border hydrolases: a) an inhibition of the post-confluent increase of activity of sucrase, aminopeptidase N and alkaline phosphatase in the brush border enriched fraction; b) an inhibition of the post-confluent increase of activity of sucrase in the cell homogenate. A comparison of the results obtained in each protocol shows that the morphological modifications and the decrease of the enzyme activities in the brush border fraction are regularly associated with an increased cAMP accumulation, whereas the inhibition of the differentiation of sucrase is a direct consequence of the increase in glucose consumption and decrease in glycogen stores.