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Pullthrough Operation for Hirschsprung's Disease: Importance of a Circumferential (Donut) Biopsy at the Level of the Anastomosis

Hirschsprung's disease is characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. It is managed by excision of the aganglionic segment and anastomosis of the ganglionated bowel just above the dentate line. The level of aganglionosis is determined by p...

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Autores principales: Jehangir, Susan, Soundappan, Soundappan Venkatraman Sannappa, Krivanek, Micheal, Arbuckle, Susan, Graf, Nicole
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Georg Thieme Verlag KG 2019
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6629996/
https://www.ncbi.nlm.nih.gov/pubmed/31312590
http://dx.doi.org/10.1055/s-0039-1693494
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author Jehangir, Susan
Soundappan, Soundappan Venkatraman Sannappa
Krivanek, Micheal
Arbuckle, Susan
Graf, Nicole
author_facet Jehangir, Susan
Soundappan, Soundappan Venkatraman Sannappa
Krivanek, Micheal
Arbuckle, Susan
Graf, Nicole
author_sort Jehangir, Susan
collection PubMed
description Hirschsprung's disease is characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. It is managed by excision of the aganglionic segment and anastomosis of the ganglionated bowel just above the dentate line. The level of aganglionosis is determined by performing multiple seromuscular biopsies and/or full thickness biopsy on the antimesenteric border of the bowel to determine the level of pullthrough. The transition zone is described as being irregular, and hence a doughnut biopsy is recommended so that the complete circumference can be assessed. Herein, we described a child in whom there was a selective absence of ganglion cells in 30% of the circumference of the bowel along the mesenteric border for most of the transverse colon. This case defies the known concept of neural migration in an intramural and transmesenteric fashion and emphasizes the importance of a doughnut biopsy of the pulled-down segment.
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spelling pubmed-66299962019-07-16 Pullthrough Operation for Hirschsprung's Disease: Importance of a Circumferential (Donut) Biopsy at the Level of the Anastomosis Jehangir, Susan Soundappan, Soundappan Venkatraman Sannappa Krivanek, Micheal Arbuckle, Susan Graf, Nicole European J Pediatr Surg Rep Hirschsprung's disease is characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. It is managed by excision of the aganglionic segment and anastomosis of the ganglionated bowel just above the dentate line. The level of aganglionosis is determined by performing multiple seromuscular biopsies and/or full thickness biopsy on the antimesenteric border of the bowel to determine the level of pullthrough. The transition zone is described as being irregular, and hence a doughnut biopsy is recommended so that the complete circumference can be assessed. Herein, we described a child in whom there was a selective absence of ganglion cells in 30% of the circumference of the bowel along the mesenteric border for most of the transverse colon. This case defies the known concept of neural migration in an intramural and transmesenteric fashion and emphasizes the importance of a doughnut biopsy of the pulled-down segment. Georg Thieme Verlag KG 2019-01 2019-07-15 /pmc/articles/PMC6629996/ /pubmed/31312590 http://dx.doi.org/10.1055/s-0039-1693494 Text en https://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Jehangir, Susan
Soundappan, Soundappan Venkatraman Sannappa
Krivanek, Micheal
Arbuckle, Susan
Graf, Nicole
Pullthrough Operation for Hirschsprung's Disease: Importance of a Circumferential (Donut) Biopsy at the Level of the Anastomosis
title Pullthrough Operation for Hirschsprung's Disease: Importance of a Circumferential (Donut) Biopsy at the Level of the Anastomosis
title_full Pullthrough Operation for Hirschsprung's Disease: Importance of a Circumferential (Donut) Biopsy at the Level of the Anastomosis
title_fullStr Pullthrough Operation for Hirschsprung's Disease: Importance of a Circumferential (Donut) Biopsy at the Level of the Anastomosis
title_full_unstemmed Pullthrough Operation for Hirschsprung's Disease: Importance of a Circumferential (Donut) Biopsy at the Level of the Anastomosis
title_short Pullthrough Operation for Hirschsprung's Disease: Importance of a Circumferential (Donut) Biopsy at the Level of the Anastomosis
title_sort pullthrough operation for hirschsprung's disease: importance of a circumferential (donut) biopsy at the level of the anastomosis
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6629996/
https://www.ncbi.nlm.nih.gov/pubmed/31312590
http://dx.doi.org/10.1055/s-0039-1693494
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