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The role of stomas in the initial and long-term management of Hirschsprung disease

      Highlights

      • Rectal washouts with primary pull-through (PT) is the preferred management for Hirschsprung's disease.
      • Colostomies are classically preferred in three-stage PT.
      • Nearly 50% of our cohort required a stoma before PT.
      • 22% required a stoma post-PT and were more likely to need a long-term stoma.

      Abstract

      Aim

      To review the role of stomas in the initial and long-term management of Hirschsprung disease (HD).

      Methods

      Patients treated for HD at our institution between January 2004 and August 2021 were identified. Data were collected regarding: demographics, indication/bowel location/type of stomas performed and outcomes, pull-through (PT) procedure, and follow-up duration.

      Results

      Ninety-five patients (78 male) were identified including one early unrelated death. Forty-four of 94 (47%) required a stoma before PT procedure. Of these 44, 38 (86%) had ileostomies and the remaining six (14%) colostomies; one ileostomy remains long-term. The commonest indication for initial stomas was washout failure (41%).
      Ninety-one patients had undergone primary PT or secondary PT with stoma closure at the time of the study.
      A further new stoma was required after primary PT or three-stage management in 20/91 (22%). The commonest indications were constipation/soiling (25%) and anastomotic leak (20%). Seven out of 20 (35%) were performed within 30 days of a previous procedure and all were closed; three patients required further long-term stomas. Thirteen (65%) required a stoma >30 days, nine remain long-term. Surgical revision of stomas was required in 14/56 (25%) – prolapse and retraction being the commonest indications. Overall, 56/94 (60%) patients required stomas (pre- and/or post-PT) to manage their condition and 13/94 (14%) have a long-term stoma in place. Mean follow-up was 7.8 years (0.5 - 17.6).

      Conclusions

      Stomas remain an integral part of HD management both initially (47%) and long-term (14%); they carry a considerable associated morbidity. Ileostomy is preferred for initial management.

      Level of Evidence

      Level III

      Keywords

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