Total colonic aganglionosis with skip lesions: report of a rare case and management
| Autor: | Ahmed Suliman, Shannon L. Castle, Barry E. LoSasso, Katayoon Shayan, Karen Kling, Stephen W. Bickler |
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| Rok vydání: | 2012 |
| Předmět: |
Male
medicine.medical_specialty Unusual case business.industry medicine.medical_treatment Colostomy Infant General Medicine Disease digestive system diseases Surgery Pediatrics Perinatology and Child Health Rare case medicine Etiology Humans Hirschsprung Disease Presentation (obstetrics) business Total colonic aganglionosis Colectomy |
| Zdroj: | Journal of Pediatric Surgery. 47:581-584 |
| ISSN: | 0022-3468 |
| DOI: | 10.1016/j.jpedsurg.2011.11.067 |
| Popis: | We present an unusual case of total colonic aganglionosis with well-documented skip lesions and discuss our staged approach for diagnosis and surgical management. To date, there have been few reported cases of total colonic aganglionosis with skip areas. This type of presentation challenges the accepted theory regarding the etiology of colonic aganglionosis. Although skip lesions in Hirschsprung disease are extremely rare, their existence must be appreciated especially when a patient's clinical and pathologic findings do not support classic Hirschsprung disease. If not considered, additional areas of aganglionosis can be missed at initial presentation, leading to a delay in definitive treatment. This case illustrates how careful mapping of bowel via multiple biopsies can identify and thereby preserve intervening segments of bowel with normal ganglions cells to yield the maximal amount of bowel possible. |
| Databáze: | OpenAIRE |
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