Reoperative techniques and management in Hirschsprung disease: a narrative review

Abstract

The majority of children who undergo operative management for Hirschsprung disease have favorable results. A subset of patients, however, have long-term dysfunctional stooling, characterized by either frequent soiling or obstructive symptoms. The evaluation and management of a child with poor function after pull-through for Hirschsprung disease should be conducted by an experienced multidisciplinary team. A systematic workup is focused on detecting pathologic and anatomic causes of pull-through dysfunction. This includes an exam under anesthesia, pathologic confirmation including a repeat biopsy, and a contrast enema, with additional studies depending on the suspected etiology. Obstructive symptoms may be due to technique-specific types of mechanical obstruction, histopathologic obstruction, or dysmotility-each of which may benefit from reoperative surgery. The causes of soiling symptoms include loss of the dentate line and damage to the anal sphincter, which generally do not benefit from revision of the pull-through, and pseudo-incontinence, which may reveal underlying obstruction. A thorough understanding of the types of complications associated with various pull-through techniques aids in the evaluation of a child with postoperative dysfunction. Treatment is specifically tailored to the patient, guided by the etiology of the patient's symptoms, with options ranging from bowel management to redo pull-through procedure. This review details the workup and management of patients with complications after pull-through, with a focus on the perioperative management and technical considerations for those who require reoperation.

Keywords: Hirschsprung disease; aganglionosis; pull-through; reoperation.

Figure 1

Anorectal manometry (ARM) tracings demonstrating the presence of a recto-anal inhibitory reflex (RAIR), wherein the pressure within the internal anal sphincter decreases (arrows) with increasing pressure applied to the rectal balloon. In a patient with Hirschsprung disease (HD), there is an absence of anal relaxation despite high rectal balloon pressure (×) (courtesy of Claudio J. Morera, MD, Boston Children’s Hospital).

Figure 2

A boy with fecal incontinence after pull-through for HD. Exam under anesthesia reveals (A) severe diaper dermatitis and (B) destruction of the dentate line from the initial pull-through, with colonic mucosa from the pull-through reaching the skin and loss of the anal canal. HD, Hirschsprung disease.

Figure 3

A child with soiling after pull-through for HD. Exam under anesthesia reveals a patulous anus (A) in the setting of a preserved dentate line (B), suggesting over-stretch and damage to the anal sphincter. HD, Hirschsprung disease.

Figure 4

A restricting aganglionic cuff (arrow) after a Soave endorectal pull-through.

Figure 5

A child with recurrent enterocolitis after a Duhamel operation. Contrast enema demonstrates a dilated “mega pouch” (A), requiring resection of the pouch (B) with redo Swenson pull-through.

Figure 6

A child with obstructive symptoms after Swenson pull-through was found to have a retained aganglionic segment (R), requiring redo pull-through and resection of this as well as dilated upstream bowel.

Figure 7

A focally dilated and dysmotile left colon in a child with a histopathologically normal pull-through and ongoing obstructive symptoms not responsive to botulinum toxin.

Figure 8

Management algorithm for patients with dysfunctional stooling after pull-through for Hirschsprung disease. *, a patient with soiling symptoms found to have overflow incontinence should undergo workup for obstructive symptoms.