Patient Case Discussion
Case Summary
A term baby with delayed meconium and bilious emesis was found to have a colonic atresia and potential Hirschsprung disease. The team deliberated on the challenges of diagnosing Hirschsprung's disease, the importance of pathology in such cases, and the findings from a biopsy. The team discussed the takedown of the anastomosis and the importance of considering Hirschsprung disease in cases of colonic atresia.
Colonic Atresia and Hirschsprung Disease Discussion
The team discussed a case involving a colonic atresia and the potential for Hirschsprung disease. The team also discussed the importance of pathology in such cases, with a pathologist sharing their experience of making a potentially incorrect call due to rushed procedures and lack of proper staining material. The team agreed that the pathology was a highlight of the case and emphasized the importance of correlation with pathology.
Challenges in Diagnosing Hirschsprung Disease
Diagnosing Hirschsprung disease is challenging, particularly in cases where the tissue sample is limited or difficult to interpret. The team acknowledged the difficulty in making a diagnosis based on frozen section analysis, especially when it comes to identifying ganglion cells. The team agreed that in cases where the diagnosis is not clear, a stoma should be considered. The conversation ended with a discussion on how to improve future diagnoses, with suggestions including taking deeper sections of tissue for analysis and expediting the process of obtaining a permanent section.
Biopsy Findings and Surgical Procedures Discussed
The team discussed the findings from a biopsy, focusing on the atrophic colon portion and the extra tissue. Highlighted was the absence of ganglion cells and large nerves in the submucosa. Also discussed was the resection of the proximal part ileocecal valve and the strangulation of the ileocecal valve. They noted the presence of ganglion cells in the appendix and the terminal ileum, as well as ulcerations and full thickness necrosis. The moderator emphasized the importance of identifying these features for pathologists and surgeons.. The case involved the takedown of the anastomosis, with no leaks identified and the presence of ganglion cells.
Hirschsprung Disease and Colonic Atresia Discussion
Others shared their thoughts on the case, emphasizing the importance of considering Hirschsprung disease in cases of colonic atresia. It was suggested that Santulli stoma should be used if a pathologist is not available. The team also discussed the gestational age at which they would start to confidently identify ganglion cells.