Search results for "Hirschsprung's disease"
showing 7 items of 7 documents
Etiopathological aspects of achalasia: lessons learned with Hirschsprung's disease
2011
SUMMARY The etiology of primary esophageal achalasia is largely unknown. There is increasing evidence that genetic alterations might play an important but underestimated role. Current knowledge of the genetic base of Hirschsprung's disease in contrast is far more detailed. The two enteric neuropathies have several clinical features in common. This association may also exist on a cellular and molecular level. The aim of this review is to enlighten those etiopathogenetic concepts of Hirschsprung's disease that seem to be useful in uncovering the pathological processes causing achalasia. Three aspects are looked at: (i) the genetic base of Hirschsprung's disease, particularly its major suscept…
Hirschsprung's disease prevalence in Europe: A register based study
2014
Background: Hirschsprung's disease is a congenital gut motility disorder, characterised by the absence of the enteric ganglion cells along the distal gut. The aim of this study was to describe the epidemiology of Hirschsprung's disease, including additional congenital anomalies, total prevalence, trends, and association with maternal age. Methods: Cases of Hirschsprung's disease delivered during 1980 to 2009 notified to 31 European Surveillance of Congenital Anomaly registers formed the population-based case-series. Prevalence rates and 95% confidence intervals were calculated as the number of cases per 10,000 births. Multilevel Poisson regression was performed to investigate trends in prev…
Complex long-segment intestinal dysganglionosis.
2000
A case is reported with aganglionosis of the rectum, sigma, and descending colon; dysganglionosis with heterotopic ganglionic cells in the muscularis propria of the hypoganglionic transverse colon; and extreme hypoganglionosis (without detection of ganglionic cells) of the ascending colon and distal ileum. The ileum showed a transition zone with hypoganglionosis and intestinal neuronal dysplasia (IND) type B. As to the etiology of such complex intestinal innervation defects, pre- and perinatal perfusion deficits must be considered because their localization seems to be linked to the vascular anatomy of the colon. Early diagnosis may be difficult, causing a delay in operative treatment and m…
Trattamento laparoscopico del megacolon agangliare
2007
Recent trends in surgery for Hirschsprung's disease have been toward early repair and fewer surgical stages. Laparoscopy can be used for colon pull-through even in neonates adding the advantages of a minimally invasive approach to those of the traditional technique. the personal experience in the use of laparoscopically assisted endorectal pull-through in the treatment of Hirschsprung's disease in reported. the advantages it offers are outlined, including excellent visualization of the operative field, accurate dissection of the bowel from pelvic structures, and minimally invasive abdominal wounds. Preliminary results show that laparoscopically assisted endorectal pull-through is one of the…
Congenital malformations and perinatal morbidity associated with intestinal neuronal dysplasia.
1998
A close relation between different forms of dysganglionosis such as intestinal neuronal dysplasia (IND) type B and aganglionosis has been established. No systematic analysis of other malformations and diseases accompanying IND has been made as yet. Congenital malformations and perinatal morbidity were analyzed in 109 patients with IND seen at the Department of Pediatric Surgery in Mainz from 1977 to 1996. IND was associated with Hirschsprung's disease in 47 cases; 22 children with IND had other abdominal malformations, including anal atresia, rectal stenosis, sigmoidal stenosis, ileal atresia, pyloric stenosis, and esophageal atresia. A cystic bowel duplication, a choledochal cyst, and a pe…
Repeated pull-through surgery for complicated Hirschsprung's disease--principles derived from clinical experience.
2007
Abstract Background In some patients, an initial pull-through procedure for Hirschsprung's disease fails, and obstructive symptoms persist or recur. Then a repeated pull-through operation may be necessary. Methods Seventeen patients with Hirschsprung's disease aged 2 to 9 years (median, 4.6 years) have undergone a repeated pull-through procedure because of unresponsive symptoms after an initial operation. The initial procedure was Soave in 3 patients, Rehbein in 13 patients, and Duhamel in 1 patient. Surgical revision was indicated by incomplete resection of the transition zone in 16 patients, anastomotic strictures in 9 patients, and fistulas in 2 patients. All 17 patients have undergone R…
Transanal coloanal anastomosis for Hirschsprung's disease: comparison between endorectal and perirectal pull-through procedures.
2006
Le but de cette etude est de comparer les resultats de 2 techniques chirurgicales d'abaissement transanal par voie perineale pour maladie de Hirschsprung de forme recto-sigmoidienne. Materiel et Methode: Vingt-et-un patients porteurs de maladie de Hirschsprung de forme recto-sigmoidienne ont ete consecutivement operes entre novembre 1999 et avril 2003 dans les services de Chirurgie Pediatrique de Dijon et Strasbourg. Les 21 enfants ont ete operes par voie perineale transanale: Douze d'entre eux ont eu une dissection peri-rectale (TPR) et 9 une endorectale type Soave (TER). Les 2 groupes de patients ont ete compares pour la longueur du segment aganglionnaire, l'âge et le poids au moment de l…