A unique type of short-segment Hirschsprung Disease, predicting a worse prognosis?

Purpose: To identify that a unique type of short-segment Hirschsprung Disease (SHD), presenting as a suddenly dilated bowel without a visible transition zone on a preoperative barium enema, have a delayed diagnosis and worse prognosis. Methods: : A comparative study was performed to analyze the clinical features and prognosis between those with a visible transition zone (type Ⅱ=69) and those without (type Ⅰ=15) on preoperative barium enema. Results: : Age at diagnosis and radical surgery in the type Ⅰ SHD group were significantly older than in the type Ⅱ SHD group (p=0.002 and p=0.001, respectively). Besides, patients with type Ⅰ SHD were more likely to suffer anastomosis leakage and postoperative enterocolitis after TEPT (p=0.017 and p=0.023, respectively). In addition, patients with type Ⅰ SHD are inclined to suffer lower bowel function scores (p=0.018). Further, we found that patients with type Ⅰ SHD were more likely to suffer poorer ability to hold back defecation (p=0.023), soiling (p=0.011), fecal accidents (p=0.004), and social problems (p=0.004). Conclusion: Compared with type Ⅱ SHD, type Ⅰ SHD is diagnosed and performed TEPT at an older age. Besides, patients with type Ⅰ SHD are inclined to suffer postoperative enterocolitis, anastomosis leakage, and poorer long-term bowel function following TEPT. Type of Study: A retrospective single-center study Level of evidence: Ⅲ