Unicentric Series of 82 Consecutive Patients With Ultralong Hirschsprung Disease: How Experience Leads Management

Total Colonic (TCSA), Extended (EA), and Total Intestinal Aganglionosis (TIA) are rare forms of Hirschsprung (HSCR) grouped under the term Ultralong HSCR. We aim at presenting the features of the largest unicentric series reported so far and at sharing the algorithm for management adopted in our Center for Digestive Diseases.

We enrolled all consecutive patients with Ultralong HSCR admitted between 2017 and 2024. Data regarding clinical features, diagnosis, management, and outcome have been recorded along with details regarding definition, classification and management.

A total of 82 patients were included, 69 being TCSA, 7 EA and 6 TIA. Length of involved ileum in TCSA ranged between 5 and 75 cm. Average age at pull-through (PT) was 2 years. Surgical complications were experienced by 36 % of patients regardless of type of PT. Postoperative enterocolitis (HAEC) were more frequent after Duhamel procedures. Stoma was fashioned 40 cm from the Ligament of Treitz (LOT) in TIA or as a levelling jejunostomy in EA. Three EA patients underwent Skipped Aganglionic Lengthening Transposition (SALT) to improve enteral autonomy with good results. Other lengthening procedures or autologous reconstructions proved to be less effective. Five patients with EA/TIA received total enterectomy nullifying the possibility for subsequent lengthening procedures. Mortality rate was 2.4 %.

Based on these results and on literature data, we propose an outcome-driven classification based on the length of ganglionated bowel below the LOT. We also suggest a management protocol aimed at improving survival for these delicate subgroups of HSCR patients.