Hirschsprung disease in adulthood: An alternative surgical approach using a U-shaped circular anastomosis

Aim-Background

Hirschsprung Disease (HD), congenital intestinal aganglionosis, or aganglionic megacolon represents a congenital abnormality which is characterized by the absence of neural crest-derived enteric neural ganglia along a variable length of the intestine. Affecting mainly children, it is an infrequent clinical entity in adulthood.

Case report

The case of a 23-year-old female with an acute faecal impaction and history of chronic constipation and long-term use of laxatives is presented. An alternative surgical technique (curvilinear stapler) for coloanal anastomosis and review of the literature are discussed.

Results

A successful U-shaped anastomosis 1.5-2 cm above the dentate line, with no postoperative stricture or incontinence as assessed by manometry is described below in this case report.

Conclusion

Proctocolectomy by curvilinear staplers represents an efficacious treatment method for aganglionosis, offering a good quality of life to patients with Hirschsprung.