Hirschsprung Disease

Case Summary

• Patient was diagnosed with Hirschsprung disease. Patient was from Nunavik, a region of Quebec in the extreme northern part, about a 1,400 km from Montreal. Patient underwent surgery at 25 days of age.
• The child had a successful recovery and was discharged.
• Patient faced difficulties with toilet training and chronic soiling issues, leading to social issues at school. Highlighted were some of the challenges of following up with patients in remote areas, as well as the efforts made by the team to provide necessary care to these patients.
• Before going back to his hometown for about 2 wk and at the end of his follow up he developed a small stenotic ring that required the dilation with Hager dilator and all of that resolved 6 mo post up
• He was followed up via the phone at 3 yr old.
• The team discussed the possibility of Hirschsprung or a long cuff structure.
• They conducted an examination under anesthesia, which revealed a retained suave cuff and a significant disruption of the dentate line with scarring, but no evidence of an astronomic structure.
• The team acknowledged that continued discussion was needed about the prognosis for patients with dentate lines damaged by scarring. They discussed the assessment and treatment plan for a child with a severely distended neorectum and 24/7 soiling.
• The question was addressed of whether to attempt a diversion or a laparoscopy-assisted pull through, concluding that given the severity of the child's symptoms, a pull through was the most appropriate option. They opted for the latter, due to the severity of the child's symptoms and the size of the cuff.
• Despite challenges in bowel management and the need for long-term care, the team proceeded with the surgery.
• The redo pull through was challenging due to the length and thickness of the cuff, but the team was ultimately able to successfully remove it.