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Reconstructing the anal sphincters to reverse iatrogenic overstretching following a pull-through for Hirschsprung disease. One-year outcomes

Published:November 06, 2022DOI:https://doi.org/10.1016/j.jpedsurg.2022.10.052

      Highlights

      • Soiling after Hirschsprung disease pull-through may be related to sphincter damage which currently has no optimal treatment.
      • A new technique of anal sphincter reconstruction showed improvement of bowel control in these patients at one year.
      • Non-sedate 3D-anorectal manometry showed improvement of squeeze function and ability to complete close the anal sphincters.
      • In patients without Down syndrome, improvement in Baylor Continence Scale was demonstrated (4.5 vs. 0.75).

      Abstract

      Background

      In patients with Hirschsprung disease (HSCR), soiling may be related to anal sphincter damage following the initial pull-through. No optimal treatment has been developed for such patients, although enemas (rectal or antegrade) have been applied with some success. We present the one-year outcomes of a new technique for anal sphincter reconstruction.

      Methods

      All patients with HSCR referred from other institutions for post pull-through soiling were studied. Seven patients with patulous sphincters underwent sphincter reconstruction. Six had a full preoperative evaluation and were included in the study. Their 12-month outcomes were assessed.

      Results

      All six patients had soiling without voluntary bowel movements (VBMs). One patient was clean on Malone flushes when referred. Three underwent pre- and post-reconstruction non-sedated three-dimensional anorectal manometry, and objectively were able to close their sphincters following the reconstruction. All patients without Down syndrome (4 of 6) showed improvement in the abbreviated Baylor Continence Scale (4.5 vs. 0.75). One patient has achieved total bowel control without antegrade flushes, three now have VBMs which they did not have before but have occasional accidents and use antegrade flushes intermittently. They reported higher productivity, the ability to participate in sports and be away from home with confidence in their regimen. Two of 6 patients have Down syndrome and required a redo pull-through for other indications and underwent empiric sphincter reconstruction. For these two patients we do not have an outcomes assessment.

      Conclusions

      A new technique for sphincter reconstruction shows promising results in improvement of bowel control at one year.

      Level of Evidence

      IV

      Keywords

      Abbreviations:

      3D HR-ARM (three-dimensional high resolution anorectal manometry), ACE (antegrade continence enema), BCS (Baylor Continence Scale), BM (bowel movement), EUA (examination under anesthesia), HSCR (Hirschsprung disease), RAIR (rectoanal inhibitory reflex), SR (sphincter reconstruction), VBM (voluntary bowel movement)
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      References

        • Burusapat C.
        • Hongkarnjanakul N.
        • Wanichjaroen N.
        • et al.
        Anorectal malformation with didelphys uterus: extremely rare anomaly and successful neoanal sphincter reconstruction with gracilis muscle flap.
        Arch Plast Surg. 2020; 47: 272-276
        • Smith C.
        • Ambartsumyan L.
        • Kapur R.P.
        Surgery, surgical pathology, and postoperative management of patients with Hirschsprung disease.
        Pediatr Dev Pathol. 2019; 23: 23-39
        • Langer J.C.
        • Rollins M.D.
        • Levitt M.
        • et al.
        Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease.
        Pediatr Surg Int. 2017; 33: 523-526
        • Bischoff A.
        • Frischer J.
        • Knod J.L.
        • et al.
        Damaged anal canal as a cause of fecal incontinence after surgical repair for Hirschsprung disease – a preventable and under-reported complication.
        J Pediatr Surg. 2017; 52: 549-553
        • Rintala R.J.
        • Pakarinen M.P.
        Outcome of anorectal malformations and Hirschsprung's disease beyond childhood.
        Semin Pediatr Surg. 2010; 19: 160-167
        • Saadai P.
        • Trappey A.F.
        • Goldstein A.M.
        • et al.
        Guidelines for the management of postoperative soiling in children with Hirschsprung disease.
        Pediatr Surg Int. 2019; 35: 829-834
        • Tan J.J.Y.
        • Chan M.
        • Tjandra J.J.
        Evolving therapy for fecal incontinence.
        Dis Colon Rectum. 2007; 50: 1950-1967
        • Halleran D.R.
        • Lu P.L.
        • Ahmad H.
        • et al.
        Anal sphincter botulinum toxin injection in children with functional anorectal and colonic disorders: a large institutional study and review of the literature focusing on complications.
        J Pediatr Surg. 2019; 54: 2305-2310
        • Soh H.J.
        • Nataraja R.M.
        • Pacilli M.
        Prevention and management of recurrent postoperative Hirschsprung's disease obstructive symptoms and enterocolitis: systematic review and meta-analysis.
        J Pediatr Surg. 2018; 53: 2423-2429
        • Ahmad H.
        • Yacob D.
        • Halleran D.R.
        • et al.
        Evaluation and treatment of the post pull-through Hirschsprung patient who is not doing well; Update for 2022.
        Semin Pediatr Surg. 2022; 31151164
        • Sun X.
        • Wang R.
        • Zhang L.
        • et al.
        Efficacy of pelvic floor muscle training for the treatment of fecal incontinence after soave procedure for Hirschsprung disease.
        Eur J Pediatr Surg. 2012; 22: 300-304
        • Levitt M.A.
        • Dickie B.
        • Peña A.
        The Hirschsprungs patient who is soiling after what was considered a “successful” pull-through.
        Semin Pediatr Surg. 2012; 21: 344-353
        • Kapur R.P.
        • Smith C.
        • Ambartsumyan L.
        Postoperative pullthrough obstruction in Hirschsprung disease: etiologies and diagnosis.
        Pediatr Dev Pathol. 2020; 23: 40-59
        • Krois W.
        • Reck C.A.
        • Darbari A.
        • et al.
        A technique to reconstruct the anal sphincters following iatrogenic stretching related to a pull-through for Hirschsprung disease.
        J Pediatr Surg. 2021; 56: 1242-1246
        • Brandt M.L.
        • Daigneau C.
        • Graviss E.A.
        • et al.
        Validation of the Baylor Continence Scale in children with anorectal malformations.
        J Pediatr Surg. 2007; 42 (discussion 1021): 1015-1021
        • Tham S.W.
        • Rollins M.D.
        • Reeder R.W.
        • et al.
        Health-related quality of life in children with Hirschsprung disease and children with functional constipation: parent-child variability.
        J Pediatr Surg. 2022; 57: 1694-1700
        • Barbosa M.
        • Glavind-Kristensen M.
        • Moller Soerensen M.
        • et al.
        Secondary sphincter repair for anal incontinence following obstetric sphincter injury: functional outcome and quality of life at 18 years of follow-up.
        Color Dis. 2020; 22: 71-79
        • Harvey M.A.
        • Pierce M.
        • Walter J.E.
        • et al.
        Obstetrical Anal Sphincter Injuries (OASIS): prevention, recognition, and repair.
        J Obstet Gynaecol Canada. 2015; 37: 1131-1148
        • Williams A.
        • Adams E.J.
        • Tincello D.G.
        • et al.
        How to repair an anal sphincter injury after vaginal delivery: results of a randomised controlled trial.
        BJOG An Int J Obstet Gynaecol. 2006; 113: 201-207
        • Lockhart-Mummery J.P.
        Diseases of the rectum & colon and their surgical treatment.
        Macmillan, Toronto Toronto1923
        • Sultan A.H.
        • Monga A.K.
        • Kumar D.
        • et al.
        Primary repair of obstetric anal sphincter rupture using the overlap technique.
        Br J Obstet Gynaecol. 1999; 106: 318-323
        • Maldonado P.A.
        • Mcintire D.
        • Corton M.M.
        Long-term outcomes after overlapping sphincteroplasty for cloacal-like deformities.
        Female Pelvic Med Reconstr Surg. 2019; 25: 271-278
        • Galandiuk S.
        • Roth L.A.
        • Greene Q.J.
        Anal incontinence - sphincter ani repair: indications, techniques, outcome. Langenbeck's.
        Arch Surg. 2009; 394: 425-433