Staged laparoscopic-assisted pull-through for Hirschsprung’s disease

Department of Pediatric Surgery, K.M. School of Postgraduate Medicine & Research, N.H.L. Municipal Medical College, V.S. Hospital, Ahmedabad, India.
Journal of Pediatric Surgery (Impact Factor: 1.39). 12/2003; 38(11):1667-9. DOI: 10.1016/S0022-3468(03)00586-4
Source: PubMed


The authors report 3 cases of Hirschsprung's disease that were treated by laparoscopic-assisted transanal pull-through after a colostomy already had been performed. Two of these patients presented with severe enterocolitis, and a primary laparoscopic-assisted single-stage transanal pull through was not feasible. The third patient had a colostomy performed and was referred to us for a definitive procedure. Many centers over the world now perform laparoscopic-assisted single-stage pull-through as a primary modality of management for Hirschsprung's disease. But for a country like India, where patients with Hirschsprung's disease present or are referred late and frequently with enterocolitis, performing a primary procedure is not possible in all cases. However, this has been used as the definitive procedure after performing a diverting colostomy and histopathologic determination of the length of the aganglionic bowel. The procedure gives excellent results and permits early postoperative feeding, early hospital discharge, and good cosmetic results.

  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Hirschsprung disease, mental retardation, microcephaly, and specific craniofacial dysmorphism were observed in three children from a large, consanguineous, Moroccan family. A fourth child showed similar clinical features, with the exception of Hirschsprung disease. The association of these abnormalities in these children represents the Goldberg-Shprintzen syndrome (OMIM 235730). Mutation scanning of genes potentially involved in Hirschsprung disease, RET, GDNF, EDN3, and EDNRB, showed a sequence variant, Ser305Asn, in exon 4 of the EDNRB gene in the index patient of this family. The Ser305Asn substitution present in two of the four patients and four healthy relatives and absent in one of the remaining two patients illustrates the difficulties in interpreting the presence of mutations in families with Hirschsprung disease. It is unlikely that the EDNRB variant contributes to the phenotype. This consanguineous family might be useful for the identification of a Goldberg-Shprintzen locus.
    Preview · Article · Jan 2005
  • [Show abstract] [Hide abstract]
    ABSTRACT: Hindgut abnormalities encompass a broad range of congenital defects. This article focuses on defects in intestinal innervation and anorectal malformations. This article also touches on suppurative diseases as they pertain to pediatric surgery. During the last several decades, significant advances have been made in the understanding and treatment of Hirschsprung's disease and intestinal neuronal dysplasia as well as in the correction of anorectal malformations. The main aim of the surgical treatment for these patients is to create a reconstructed anatomy that functions as close to normal as possible, while treating the functional sequelae of these defects in an attempt to provide these children with a good quality of life.
    No preview · Article · May 2006 · Surgical Clinics of North America
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Neural crest (NC) cells differentiate IN VITRO into neuroblasts, precursors of the enteric nervous system (ENS), when stimulated by specific agents. We developed a study aimed at establishing whether NC-derived neuroblasts can survive and colonise IN VIVO when injected into a recipient mouse gut. The neuroblast precursors of the ENS were obtained from the vagal portion of the neural tubes of 296 CD-1 and GTROSA26 mouse embryos. The embryonic cells of GTROSA26 mice are identifiable through beta-galactosidase activity which allows recognition by blue staining. The host used in this study was the DOM/+ mouse, an animal model for Hirschsprung's disease (aganglionic megacolon). DOM/+ mouse pups (n = 43) received NC-derived cells inoculated into the seromuscular layer of the gut (33/43) or directly into the peritoneal abdominal cavity (10/43). All DOM/+ mice survived the procedure and were sacrificed after 7 or 14 days. Histochemical staining detected implanted cells in all mice. These showed specific myenteric colonisation into the aganglionic and ganglionic gut. The striking result of this study was the specific tropism of the injected NC-derived cells to target sites under the action of unknown chemotactic agents. This experimental procedure might represent a possible treatment option for specific forms of human ENS anomaly such as total intestinal aganglionosis.
    Full-text · Article · Mar 2007 · European Journal of Pediatric Surgery
Show more