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.

[Hematemesis in an 11-month-old child: an unusual symptom revealing a foreign body in the stomach]. / Hématémèse chez un enfant de 11 mois: un mode de révélation rare d'un corps étranger intragastrique.

Ingestion of foreign body has often no consequence. We report on a case in an 11-month-old girl who was referred for mild hematemesis and anorexia. Upper digestive tract endoscopy found a small metallic foreign body in the gastric antrum. After its removal, all symptoms disappeared. It is usually recommended to remove foreign bodies by endoscopy when they are in esophageal position, or are more than 3 to 5 cm long, or have a shape that may hurt the gut mucosa. Although rare, a gastric foreign body should be searched for in face of an upper gastrointestinal bleeding in an infant.

Reevaluation of the relative risk for susceptibility to celiac disease of HLA-DRB1, -DQA1, -DQB1, -DPB1, and -TAP2 alleles in a French population.

In a population of 46 children with CD recruited in the Paris area of France, an excess of DRB1*03 and DRB1*07 alleles and of DR3/DR7, DR3/DR3 and DR11(or 12)/DR7 phenotypes was found (RRs of 6.3, 9.3, 24.6, 15, and 15.1, respectively), which is reminiscent of the markers of susceptibility observed in southern rather than in northern European celiac patients. More importantly, the highest association with CD was not found in individuals expressing the DQA1*0501-DQB1*0201 heterodimer in single dosage (RR = 24.9) or in homozygous state, but in people co-expressing one copy of DQA1*0501-DQB1*0201 on one haplotype and a second copy of DQB1*0201 on the second haplotype (RR = 35.7). This suggests that in our population either DQB1*0201 or a gene closely linked to DQB1*0201 influences the susceptibility to CD conferred by the DQA1*0501-DQB1*0201 heterodimer. Significant positive or negative RRs conferred by some TAP2 or DPB1 alleles were found. However, they were moderate compared to the RR conferred by the expression of a second copy of DQB1*0201. Moreover, they were no longer significant when patients were compared with HLA-DR matched controls. This suggests that associations of CD with TAP2 and DPB1 alleles are secondary to linkage disequilibria and argues against the contribution of these alleles in resistance and/or susceptibility to CD. Thus the "raison d'être" of a "DQB1*0201 second haplotype effect" in susceptibility to CD remains to be elucidated.

Lansoprazole in children: pharmacokinetics and efficacy in reflux oesophagitis.

BACKGROUND: Data on the proton pump inhibitor lansoprazole in paediatric patients are limited. AIM: To investigate the pharmacokinetics, optimal dosage and efficacy of lansoprazole in paediatric patients. METHODS: A 24-h gastric pH recording and a pharmacokinetic study were performed after 7 days of lansoprazole, 17 mg/m2, in 23 patients with reflux oesophagitis (median age, 3.5 years). Response was defined as pH > 3 for > 65% of the recording. The dosage was doubled in non-responders. Patients with no response on day 14 were excluded. Responders underwent endoscopy after 4 weeks on the response-inducing dosage; abnormal findings led to a repeat endoscopy after four additional weeks. RESULTS: Nine patients responded to 17 mg/m2 and six to 30.3 mg/m2. On day 7, time with pH > 3 was significantly correlated with the area under the plasma concentration-time curve (P=0.003). The area under the plasma concentration-time curve was significantly greater in the nine responders to 17 mg/m2 than in the 14 other patients. Pharmacokinetic parameters were similar in responders and non-responders to the higher dose. After 4 weeks, oesophagitis was healed in 80% of responders. Adverse events occurred in three patients and required treatment discontinuation in one. CONCLUSIONS: Lansoprazole is effective and safe in children. The optimal starting dosage is 30 mg/m2 or 1.4 mg/kg.

Collagenous gastritis revealed by severe anemia in a child.

Collagenous gastritis is a rare histopathological disorder of unknown origin, characterized by a subepithelial collagen deposit greater than 10 microm thick, associated with an inflammatory infiltrate of the gastric mucosa. This report describes a second pediatric case of collagenous gastritis, revealed by severe anemia caused by gastric bleeding, as was the first case. Unlike the adult cases of collagenous gastritis, lesions were limited to the stomach, and remained unchanged on six series of biopsies taken during a 30 month follow-up, despite treatment with omeprazole, sucralfate and corticosteroids. An immunohistochemical study showed signs of local immune activation on all biopsy specimens, including overexpression of HLA-DR by epithelial cells, increased numbers of CD3+ intraepithelial lymphocytes, and CD25+ cells in the lamina propria. Although the cause of the disease remains unclear, our findings suggest that the histopathological lesions of collagenous gastritis may result from a local immune process.

Early diagnosis of adenovirus infection and treatment with cidofovir after bone marrow transplantation in children.

Adenovirus infection remains an important cause of mortality after bone marrow transplantation (BMT). Currently no efficient antiviral treatment is known. Thus, testing new modalities of early diagnosis and treatment is a crucial objective. Adenovirus infection is defined by the combination of symptoms and the isolation of virus from the source of clinical symptoms. The involvement of two or more organs and the presence of virus in blood cultures define disseminated disease. Seven children with a median age of 7 years received bone marrow transplantation for leukemia. All received an unrelated graft without T cell depletion. Adenovirus was sought in blood, urine and biopsy specimens using PCR and culture. Analysis of biopsy specimens included systematic immunohistochemistry. Cidofovir treatment was initiated as soon as biopsy revealed the histopathological signs of adenovirus. Cidofovir was given at 5 mg/kg once weekly for 3 weeks then every 2 weeks. Six patients had diarrhoea and one patient had cystitis. Adenovirus infection and disseminated disease were diagnosed in four cases and three cases, respectively. In six cases, serotype A31 was isolated from gastrointestinal biopsy and in two cases serotypes B2 and C6 were detected in blood and urine. Cidofovir treatment was associated with clinical improvement of diarrhoea, cystitis and fever in five patients, in whom the virus became undetectable in cultures and PCR analyses despite the persistence of immunodeficiency. The median follow-up was 360 days after BMT (240-570). One child died of invasive aspergillosis and another of disseminated adenovirus after interruption of cidofovir therapy. Further studies in immunocompromised patients will be needed to extend these promising results concerning the role of cidofovir in adenovirus infection.

[Esophageal dilatation in pediatrics: study of 33 patients]. / Les dilatations oesophagiennes en pédiatrie: étude de 33 patients.

BACKGROUND: Esophageal dilatation is usually regarded as an effective therapy in a majority of esophageal stenosis in childhood. However, the limited number of pediatric data does not allow definite conclusions on indications and complications of such a procedure. PATIENTS AND METHODS: The files of 33 children whose esophageal stenosis had been treated by dilatation by the same operator between 1983 and 1992 were retrospectively reviewed. The structure mechanisms were: group 1: repair of esophageal atresia (n = 9), group 2: caustic esophagitis (n = 6), group 3: peptic esophagitis (n = 12), group 4: unclassified structures (congenital esophageal stenosis, achalasia) (n = 6). The dilatations were performed under general anesthesia, and the dilatator guide was introduced under endoscopic control. Two methods were used: Savary esophageal bougies and balloon dilatation. A thoracic X-ray was systematically performed after each dilatation. RESULTS: One hundred and fourteen dilatations (3.5 dilatations/child) were performed (range: 1-32 dilatations). Twenty-five of the 33 children (76%) were dramatically improved after mechanical dilatation. Esophageal dilatation was unsuccessful in the eight other patients, seven of them requiring a surgical repair. Complications occurred in 3.4% of the dilatations: one esophageal perforation, one pneumomediastinum and two cardiac arrests (one of vagal origin and 1 after accidental extubation). All patients survived. Efficacy, duration of dilatation and complication rates were not similar in the four groups. CONCLUSIONS: Esophageal dilatation should be considered as a simple and effective procedure when strict security rules are respected by a trained operator.

[Pediatric gastrointestinal endoscopy: which sedation?]. / Endoscopie digestive pédiatrique: quelle sédation?

Through an analysis of the French experience of digestive endoscopists in adult patients and of their own 25 years practice of pediatric digestive endoscopy, the authors militate in favour of anesthetic sedation in order to reduce painfulness and to obtain better acceptation of these procedures by children and their parents.

[Adenocarcinoma on villous tumor of the rectum in an adolescent]. / Adénocarcinome sur tumeur villeuse du rectum chez un adolescent.

CASE REPORT--A 15-year 6-month-old boy suffered from isolated recurrent rectal bleeding. Rectoscopy and colonoscopy allowed to identify and to excise one large size villous polyp, which was the site of dysplasia and liberkühn adenocarcinoma. Transrectal ultrasonography showed thickened mucosa and submucosa and suspect adenopathy. Proctectomy, ganglionic curage and coloanal anastomosis were then performed; one of the excised adenopathies was metastatic. The patient died twelve hours after surgery from an unexplained cardiovascular collapse. CONCLUSIONS--Unspecific symptoms of colorectal cancer explain that its diagnosis is often delayed. Prognosis is poor related to the advanced stages at diagnosis and mucinous adenocarcinomas. Better knowledge of the clinical presentations and of high risk situations as polyposis, ulcerative colitis, hereditary non polyposis colorectal cancer could improve the prognosis.

[Perineal lesions in childhood Crohn disease]. / Les lésions anopérinéales dans la maladie de Crohn de l'enfant et de l'adolescent.

BACKGROUND: Perineal lesions (PAL) usually evolve together with bowel disease and often constitute a serious and disabling complication of Crohn's disease. PATIENTS: Forty-three children (47%) with Crohn's disease developed PAL in a retrospective study of 92 patients ranging in age from 4 to 20 years. RESULTS: PAL occurred at the mean age of 11.4 +/- 0.7 years, prior to diagnosis in 25% or subsequently in 21%. PAL were severe: complex fistulae (15%), rectovaginal fistulae (2%), anal raggedness (13%); moderate: subcutaneous fistulae (11%), abscesses (19%), cavitating ulcers (9.5%) and stricture formations (5.7%); or mild: eczema (6.7%), fissures (57%) and skin tags (17%). An association with these various features has been observed in 31%. The extent of involvement of the gastrointestinal tract was rectosigmoid (72%), ileal and colonic (41%), ileal with pancolitis (12%). Two PAL course profiles were observed: one with exacerbation and remissions (52%), the other without remission (48%), especially anal raggedness (100%), cavitating ulcers (80%) and skin tags (61%). Medical treatment included steroids (54%), metronidazole (53%), salicylates (51%), nutritional support (44%), azathioprine (17%). PAL healed in 41%. Surgical treatment was performed in 27% with 83% of healing. Relapses occurred in 35% after medical treatment and 86% after surgery.

[Long-term followup of primary intestinal lymphangiectasia in the child. Six case reports]. / Suivi de long cours des lymphangiectasies intestinales primitives de l'enfant. A propos de six cas.

UNLABELLED: Primary intestinal lymphangiectasia induce symptoms of protein-losing gastroenteropathy. Only very few studies evaluate the long term follow up of such patients. We reviewed six children diagnosed at 17 +/- 12 months and followed for 11 +/- 4.9 years. CASE REPORTS: As soon as the diagnosis was made the patients were submitted to a strict low fat diet with added medium chain triglycerides and intermittent liposoluble vitamins perfusions. The diet allowed the disappearance of symptoms for all the patients but laboratory findings indicated continuing chyle leak for most of the children. Only one child who had normal biological parameters tolerates a normal diet since four years. Relaxation of the diet by two patients who had moderate hypoalbuminemia and lymphopenia led to severe clinical relapses 14 and 17 years after the diagnosis period with therapeutic difficulties. Three patients with long term strict low fat diet remain asymptomatic. CONCLUSION: In most asymptomatic patients, the underlying lymphatic defect remains with permanent biological abnormalities. Clinical relapses may be severe and difficult to treat; thus the need for dietary treatment appears to be permanent.

[Contribution of endosonography to the diagnosis and follow-up of pediatric gastric neurofibroma revealing von Recklinghausen's disease]. / Contribution de l'échoendoscopie au diagnostic et au suivi d'un neurofibrome gastrique pédiatrique révélateur d'une maladie de von Recklinghausen.

BACKGROUND: Gastrointestinal involvement in von Recklinghausen's disease (RD) is rare during childhood; its symptoms are late and its prognosis is poor, related to local recurrence and risk of malignant transformation. CASE REPORT: A 13 year-old boy was admitted for hematemesis revealing gastric ulcer. A second episode of hematemesis led to identify a sessile gastric tumor in this patient having numerous skin café-au-lait spots. Recurrent bleeding required laparotomy that showed diffuse infiltration into the anterior gastric wall: histological examination of the excised piece showed characteristic features of neurofibromatosis. The patient was not compliant to the endosonographic survey so that a symptomatic relapse led to total gastrectomy: histological examination did not show malignant transformation. CONCLUSION: Endoscopy is a major tool for identifying gastrointestinal localization of RD but endosonography is necessary to precise the extent of the tumor.

[Pancreatic involvement in inflammatory bowel diseases in children]. / Atteintes pancréatiques dans les maladies inflammatoires du tube digestif de l'enfant.

UNLABELLED: Pancreatitis in inflammatory bowel disease (IBD) in children is anecdotal. In adults, symptomatic pancreatitis occurs in 2% and asymptomatic in 8 to 21%. PATIENTS AND METHODS: The aim of our study was to review retrospectively the frequency of pancreatitis in 124 pediatric patients (54.8% boys, 45.2% girls; 97 with Crohn disease, 16 with ulcerative colitis and 11 with undetermined colitis). Diagnostic criterion of pancreatitis was an increase of amylasemia > or = +2 SD of the normal with or without evocative clinical symptoms. RESULTS: Symptomatic or asymptomatic pancreatitis was found in 27% (respectively 14.5 and 12.5%). Pancreatitis was significantly more frequent in girls as compared to boys (P = 0.04). Symptomatic pancreatitis was moderate and non complicated, often recurrent. It occurred mainly during active and severe diseases (P = 0.006). The localizations of IBD were not discriminant. Strong relation with drug was found in 25% of pancreatitis mainly due to azathioprine or 5-aminosalicylic acid, and salazopyrin. Duodenal localisation of Crohn disease or hepatobiliary complications were found associated with pancreatitis in 18% and 15% respectively. CONCLUSION: These data suggest the high incidence of symptomatic and asymptomatic pancreatitis in children with IBD, the importance of its regular monitoring but also its multifactorial causes. Precise diagnosis of pancreatitis in pediatric IBD has to be done in order to avoid inappropriate drug pancreatitis diagnosis.

[A rare cause of rectal hemorrhage in children: rectal angiodysplasia]. / Une cause rare de rectorragie chez l'enfant: l'angiodysplasie rectale.

BACKGROUND: Parietal vascular malformations of the intestinal tract are rare and their diagnosis is often difficult. CASE REPORT: A 2 year-old boy had suffered from intermittent rectal bleeding since the age of one year. Endoscopic examination showed unspecific congestive changes and biopsy showed features of chronic inflammatory changes in the rectum. Barium enema was normal. The patient was operated on, but surgery failed to find any cause for these hemorrhages. Rectal bleedings recurred, some of them resulting in severe anemia. Two further endoscopic examination were grossly negative as was inferior mesenteric arteriography. Spontaneous rectal prolapsus occurred when the boy was 4 years old. This allowed resection of all the submucosal veins of the anal canal; it was followed by complete cessation of rectal bleeding with a follow-up of 36 months. Histological examination showed several ectasias of the capillaries and veins in the submucosa and muscular layers. CONCLUSION: Angiectases can occur in a small part of the intestinal tract, and can escape detection by repeated specialized investigation.

[Contribution of genetic typing for the diagnosis of familial adenomatous polyposis in pediatrics]. / Apport du typage génétique pour le diagnostic de polypose adénomateuse familiale en pédiatrie.

BACKGROUND: The gene responsible for familial adenomatous polyposis, (APC), has been recently cloned and genetic map with several polymorphic markers has been established. POPULATION AND METHODS: Blood samples (20 ml) were taken from 34 subjects belonging to four families at risk for familial adenomatous polyposis. Nineteen of these 34, less than 20 years old, had one parent having polyposis or dead because of it. Polyposis was diagnosed, in ten of these 19 by endoscopy. Genomic DNA was extracted from peripheral leukocytes and Southern blot analyses were performed in each family, using RFLPs on both sides of the APC locus. RESULTS: DNA analysis identified normal and mutant haplotypes at the APC locus in each family. It was thus possible to follow the segregation of mutant alleles. These results were compared with the anamnestic and endoscopic data. Bearing in mind the risk of recombination when using extragenic markers, RFLPs allowed early diagnosis of APC in pre and/or asymptomatic patients. CONCLUSIONS: Genetic analysis can be used to diagnose APC in affected families, provided the risk of recombination is taken into account. Intragenic microsatellites markers will soon be available. These will provide more information on the APC gene, and hence direct molecular diagnosis of APC.

[Iterative intestinal intussusception and appendiceal mucocele in an infant with mucoviscidosis]. / Invaginations intestinales itératives et mucocèle appendiculaire chez un enfant atteint de mucoviscidose.

UNLABELLED: Cystic fibrosis is a common and potentially life-threatening hereditary disease which can affect numerous organs, particularly the digestive tract. CASE REPORT: A 4.5-year-old boy exhibited two little known clinical manifestations: an appendiceal mucocele and repeated intussusceptions. In spite of an appendectomy, intussusception relapsed and an ileocolic resection was necessary 2 years later. DISCUSSION: Appendiceal diseases in cystic fibrosis represent a large spectrum, ie, distention on the appendiceal lumen, engorged with sticky mucous matter, which becomes an appendiceal mucocele, peritonitis with an appendiceal perforation due to delayed diagnosis since acute appendicitis is difficult to diagnose in these patients. Intussusception is rarely observed in cystic fibrosis. CONCLUSION: Appendiceal mucocele could be a cause of intussusception. If an appendectomy is performed, resection of a part of the cecum, around the appendix, could be useful in preventing again mucocele formation.

[Incidence of symptomatic celiac disease in French children]. / Incidence de la maladie coeliaque symptomatique de l'enfant en France.

OBJECTIVE: The study was carried out by the GFHGNP to determine the annual incidence of symptomatic celiac disease in children. PATIENTS AND METHODS: The diagnostic criteria were: symptomatic patients diagnosed under 15 years of age during 1996, villous atrophy and positivity of antigliadin and/or other antibodies. Cases were collected from referral centers, general hospital pediatric departments and private pediatricians with endoscopic practice. RESULTS: The study involved roughly half of the French pediatric population in 41 out of the 95 French districts. In all, 124 patients were collected: 76 girls and 48 boys. By geographical areas, in 30 districts where collection of data was complete which counted 186,285 births, the yearly incidence varied from 1/1731 births to 1/3110. (0.57@1000 to 0.32@1000). On the whole there were 77 cases i.e. an annual incidence of 1/2419 or 0.41@1000 (confidence interval 95%: 0.32 to 0.50@1000). Lower incidences were observed in the district of Paris: 1/4865 (0.21@1000) and Lyon: 1/3310 (0.27@1000). Those lower incidences could be explained by the difficulties of collecting the data in the biggest urban areas. The first signs occurred before one year of age in 73% of the cases, during the second year of life in 20.5% and after 3 in only 6.5%. The diagnosis was made before 2 years of age in 77% of the cases and after 3 in only 13%. In order of frequency symptoms were: failure to thrive (80%), diarrhea (59%), anorexia (59%), abdominal distension (57%), weight under 2 standard deviations (43%), short stature (43%). CONCLUSION: Compared with previous studies in two French districts between 1975 and 1990, the annual incidence of symptomatic celiac disease in children appears to be on the rise. The usual clinical signs continue to be observed.

[Capsule endoscopy in children: which are the best indications?]. / Quelles indications pour l'endoscopie du grêle par vidéocapsule en pédiatrie?

BACKGROUND AND STUDY AIMS: Capsule endoscopy (CE) is a novel and noninvasive means of investigating the small bowel. In children, the best CE indications have not yet been fully appraised. The aim of this study was to evaluate the diagnostic yield of CE in different pediatric pathologies. PATIENTS AND METHODS: We retrospectively reviewed every CE performed in children in two French pediatric hospitals between March 2002 and June 2009. Seventy-nine CEs were performed on 70 children (mean age, 10.6 years; range, 2.2-18.0); 52 boys and 18 girls. The indications were iron deficiency anemia (24%), obscure gastrointestinal bleeding (14%), polyposis syndromes (16%), suspected Crohn disease (15%), unresponsive Crohn disease (10%), graft-versus-host disease (10%), and other (10%). RESULTS: Of the 79 CEs, 69 reached the cecum (87%). Only one occlusion occurred in a case of stenosing Crohn disease, requiring surgical removal. In addition, technical difficulties led to an incomplete small bowel study in 12 cases (16%). The CE showed small bowel lesions in 42 cases (53%). The diagnostic yield was 27% in obscure gastrointestinal bleeding, 37% in iron-deficiency anemia, 42% in suspected Crohn disease, 88% in unresponsive Crohn disease, 62% in polyposis syndromes, and 88% in graft-versus-host disease. CONCLUSION: In children, CE is well tolerated and can be performed in children as young as 2.2 years of age. Its diagnostic yield is highest in polyposis syndromes, unresponsive Crohn disease, and graft-versus-host disease.