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...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Georg Thieme Verlag KG
2019-01-01
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| Series: | European Journal of Pediatric Surgery Reports |
| Subjects: | |
| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1693494 |
| _version_ | 1828835593988079616 |
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| author | Susan Jehangir Soundappan Venkatraman Sannappa Soundappan Micheal Krivanek Susan Arbuckle Nicole Graf |
| author_facet | Susan Jehangir Soundappan Venkatraman Sannappa Soundappan Micheal Krivanek Susan Arbuckle Nicole Graf |
| author_sort | Susan Jehangir |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-12T18:05:47Z |
| format | Article |
| id | doaj.art-18e4ee52c59a4ad9bd1ac53b51b17cf4 |
| institution | Directory Open Access Journal |
| issn | 2194-7619 2194-7627 |
| language | English |
| last_indexed | 2024-12-12T18:05:47Z |
| publishDate | 2019-01-01 |
| publisher | Georg Thieme Verlag KG |
| record_format | Article |
| series | European Journal of Pediatric Surgery Reports |
| spelling | doaj.art-18e4ee52c59a4ad9bd1ac53b51b17cf42022-12-22T00:16:30ZengGeorg Thieme Verlag KGEuropean Journal of Pediatric Surgery Reports2194-76192194-76272019-01-010701e55e5710.1055/s-0039-1693494Pullthrough Operation for Hirschsprung's Disease: Importance of a Circumferential (Donut) Biopsy at the Level of the AnastomosisSusan Jehangir0Soundappan Venkatraman Sannappa Soundappan1Micheal Krivanek2Susan Arbuckle3Nicole Graf4Department of Paediatric Surgery, Christian Medical College, Vellore, IndiaDepartment of Paediatric Surgery, The Children's Hospital at Westmead, Sydney, New South Wales, AustraliaDepartment of Anatomical Pathology, The Children's Hospital at Westmead, Sydney, New South Wales, AustraliaDepartment of Anatomical Pathology, The Children's Hospital at Westmead, Sydney, New South Wales, AustraliaDepartment of Anatomical Pathology, The Children's Hospital at Westmead, Sydney, New South Wales, AustraliaHirschsprung'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.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1693494atypical aganglionosishirschsprung's diseaseskip lesionszonal aganglionosis |
| spellingShingle | Susan Jehangir Soundappan Venkatraman Sannappa Soundappan Micheal Krivanek Susan Arbuckle Nicole Graf Pullthrough Operation for Hirschsprung's Disease: Importance of a Circumferential (Donut) Biopsy at the Level of the Anastomosis European Journal of Pediatric Surgery Reports atypical aganglionosis hirschsprung's disease skip lesions zonal aganglionosis |
| 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 |
| topic | atypical aganglionosis hirschsprung's disease skip lesions zonal aganglionosis |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1693494 |
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