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A technique to reconstruct the anal sphincters following iatrogenic stretching related to a pull-through for Hirschsprung disease

Open AccessPublished:December 12, 2020DOI:https://doi.org/10.1016/j.jpedsurg.2020.12.007

      Abstract

      Soiling and fecal incontinence are troubling complications which can occur after a pull-through for Hirschsprung disease. They can usually be improved with proper medical management, but in some cases are the result of an anatomic defect related to overstretching of the sphincters and/or damage of the anal canal. For such patients the treatment of this true fecal incontinence is limited to a structured bowel management program with ante- or retrograde enemas to achieve social continence. Herein we report two such patients with overstretched sphincters and loss of the dentate line after an initial pull-through and describe a sphincter tightening technique to improve bowel control.

      Keywords

      Abbreviations:

      HD (Hirschsprung disease), SNM (sacral neuromodulation), TNS (tibial nerve stimulation), 3-D-HR-ARM (Three-dimensional high-resolution anorectal manometry)

      Introduction

      Soiling and fecal incontinence are devastating complications of a pull-through in patients with Hirschsprung disease. In most cases, proper medical management improves the patient, but these symptoms may relate to how well the original surgery did in preserving the internal and external sphincters and the propioceptive area, dentate line [
      • Rintala RJ
      • Pakarinen MP.
      Outcome of anorectal malformations and Hirschsprung's disease beyond childhood.
      ], although the dentate line's role in continence for such patient has not been proven experimentally. An important factor but one not well understood seems also to be distension of the neorectum within the pelvic sphincters - a concept called proprioception. In addition, the pudendal nerve innervation of the anal sphincter can play an important role as well [
      • Springall RJ
      • Kiely EM
      • Boyd SG.
      The nature of neurogenic damage to the external anal sphincter in children treated for Hirschsprung's disease.
      ]. In the workup of those patients, it is essential to identify the cause of their symptoms. Abnormal sensation, inadequate sphincter control, and fast or slow colonic motility, can lead to soiling and fecal incontinence following a pull-through procedure [
      • Saadai P
      • Trappey AF
      • Goldstein AM
      • Cowles RA
      • De La Torre L
      • Durham MM
      • et al.
      Guidelines for the management of postoperative soiling in children with Hirschsprung disease.
      ].
      Soiling in the presence of an intact continence mechanism (intact sphincters and dentate line) can result from severe constipation or hypermotility, and can improve with adequate medical treatment, usually laxatives to treat constipation, or treatment of hypermotility, as well as treatment of non-relaxing sphincters with botulinum toxin [
      • Halleran DR
      • Lu PL
      • Ahmad H
      • Paradiso MM
      • Lehmkuhl H
      • Akers A
      • 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.
      ]. However, patients with a damaged anal canal are unable to sense stool consistency and distension of the neorectum which can lead to true fecal incontinence. This is believed to be a consequence of the loss of the transitional epithelium by iatrogenic resection or damage of the dentate line (i.e. when the original surgeon started the pull-through too low). Also, inadequate sphincter control due to overstretching of the sphincter mechanism during the pull-through (with injury to both the internal and external sphincters) or due to myectomy or sphincterotomy can lead to true fecal incontinence.
      To address these anatomic problems causing incontinence, we report our management of two patients with Hirschsprung disease and describe a novel idea, a sphincter tightening technique that might allow such patients to achieve bowel control. We were intrigued by this possible application for such Hirschsprung's patients, given the data in the literature on surgical treatment for anal sphincter injury in adults. Techniques for sphincteroplasty were introduced in the 1970ies and are performed by mobilization and overlapping the sphincter muscles [
      • Parks AG
      • McPartlin JF.
      Late repair of injuries of the anal sphincter.
      ,
      • Slade MS
      • Goldberg SM
      • Schottler JL
      • Balcos EG
      • Christenson CE.
      Sphincteroplasty for acquired anal incontinence.
      ,
      • Pescatori LC
      • Pescatori M.
      Sphincteroplasty for anal incontinence.
      ]. In contrast to the original sphincteroplasty, dissection of fibrous scar tissue and the sphincter mechanism's broad mobilization was not necessary for our patients.
      We specifically focused on improving the sphincters, understanding that there was no way to repair or replace a lost dentate line, but hypothesizing that sphincter enhancement might be just enough to provide bowel control to such patients.

      Case 1

      A two-year-old boy with a history of a transanal pull-through at three months of age for Hirschsprung disease had a transition zone in the sigmoid colon. After surgery, the patient suffered from a severe but spontaneously retracting, rectal prolapse of up to six centimeters. At the time of our assessment, when he was referred to us from another institution at the age of 25 months, the anal canal was absent, and the sphincters were patulous with no tone. The colonic tissue was friable and swollen due to the frequent prolapses. The appearance of the anus is demonstrated in Fig. 1a. It shows the patulous sphincters and the absent dentate line, but not the full extent of the prolapse he regularly experienced. The patient also had constant soiling and increased stool frequency. The stool frequency improved after the establishment of an initial bowel-management program with loperamide and daily rectal enemas with 300ml saline for several months preoperatively.
      Fig. 1:
      Fig. 1Anal appearance of patient 1. Patulous sphincters and the absent dentate line (a) intraoperative situs showing resection of the prolapsed colon and two tightening sutures applied at the anterior sphincter (b) image of the anus two months postoperative (c)

      Case 2

      A 17-year-old male with Hirschsprung disease was treated as a newborn with a pull-through of his right colon. We first met the patient 10 years ago when he was referred to us from another institution for management of soiling. We found his dentate line absent and his anal sphincters patulous (Fig. 2a). We placed him on daily enemas, which worked for many years to achieve social continence, however, when he became a teenager, he stopped doing them and chose to tolerate daily soiling. On re-evaluation, we had considered a Malone appendicostomy. However, his contrast enema showed that his colon was too short for this procedure. Because of the success with the first patient we offered him the sphincter reconstruction. For this patient a 3D anorectal manometry was performed prior to and after the sphincter reconstruction.
      Fig. 2:
      Fig. 2Anal appearance of patient 2. Patulous sphincters and the absent dentate line (a) intraoperative situs showing resection of the prolapsed colon and tightening sutures applied at the posterior sphincter (b) image of the anus immediately postoperative (c)

      Operative technique

      We performed a redo of the pull-through in both patients. The patients were placed in a prone position, and the anal area was exposed using the LoneStar-retractor. As noted, no dentate line was evident and even awake the sphincters appeared patulous. After placing circumferential holding-sutures in the mucosa a dissection at the skin level (at the initial anastomosis level) was performed and continued as a full-thickness dissection of the rectal wall, paying attention not to harm the sphincter muscles, just as in an original pull-through maneuver. In patient 1, with the rectal prolapse, we mobilized six centimeters of prolapsing colon from the previous pull-through. After full circular mobilization, the sphincter-repair maneuver was initiated: we placed several long-term absorbable sutures in the anterior and posterior part of the external sphincter muscle, about 1 cm apart, and at depths of 4, 3, 2, and 1 cm from the anal edge, starting from the deep planes with the first posterior stitch through to the serosal rectal wall. By placing several sutures anterior and posterior a tightening of the external sphincter around the anal canal was established. This technique is shown in Fig. 3a+b. Care was taken not to tighten too much and thus create a stricture, and the lumen was checked with digital exam and with Hegar dilators (a size 16 Hegar). The repair was completed by performing an anocutaneous anastomosis circumferentially placing interrupted stitches. At the operation's conclusion, the anus appeared closed and tight with the sphincter muscles enveloping the neo-anus. One month after the repair, the anus of patient 1 looked closed on clinical inspection with no prolapse, and the patulous appearance had disappeared (Fig. 1c). His bowel management needs changed with rectal enemas no longer required. So far, the colonic hypermotility remains but he has started to demonstrate bowel control. We are aware that the patient is too young to predict further fecal continence. Follow-up is performed with digital exam and checking of patency with Hegar dilator at 3, 6 and 12 months. We expect to perform a 3D high resolution anorectal manometry (3-D HR-ARM) on this patient in the future and are following closely for the developing of continence. It appears the child already is able to hold in the stool much better than previously and there is no recurrence of the prolapse.
      Fig. 3:
      Fig. 3Illustration of the sphincter tightening-technique: several long-term absorbable sutures in the anterior and posterior part of the external sphincter muscle, about 1 cm apart, and at depths of 4, 3, 2, and 1 cm from the anal edge, starting from the deep planes with the first posterior stitch through to the serosal rectal wall, demonstrated in sagittal view (a) and tightening (red arrows) of the external sphincter around the anal canal demonstrated in transversal view (b)
      In the second patient, the same technique (Fig. 2b) was performed, but less (1 cm) neo rectal resection was needed (as there was no prolapse, and the only goal of the intervention was for sphincter tacking to the bowel). In this case, a preoperative 3-D HR-ARM (Fig. 4a) was performed to assess the structural and functional integrity of the anal sphincters (Medtronic USA). The 3D-HR-ARM probe has 256 pressure sensors on 16 lines, each line having 16 circumferential sensors. The probe, covered with a disposable sheath, has a diameter of 10.75 mm, length of 64 mm and a 3.3 cm long balloon with a capacity up to 400 cm3. The pre-operative study showed sphincter hypotonia and weak squeeze efforts. One month after the sphincter repair, a repeat study demonstrated higher resting sphincter pressures and improved (stronger and more symmetric) squeeze effort (Fig. 4b). The patient has normal bowel control with no accidents for the first time in his life, at six-month follow-up. We anticipate that sphincter strength will continue to improve over time, and plan to recheck with another 3-D HR-ARM at a year following surgery.
      Fig. 4
      Fig. 4Three-dimensional high resolution anorectal manometry (3-D HR-ARM) performed before (a) and one month after (b) the surgical procedure; Sphincter hypotonia visualized on the left contour plot as a band of pale green (~20 – 25 mmHg resting pressures), with squeeze efforts noted in the center 3-D reconstruction and right pressure plots. a shows pre-operative tracings with sphincter hypotonia (~15 ~20 mmHg), weak and asymmetric squeeze efforts. b demonstrates post-operative measurement, with improved resting sphincter pressures (~25 ~30 mmHg) and improved squeeze efforts (more circumferential and symmetrical, compared to a) [Normal range is between 60 to 74 mmHg]

      Discussion

      A damaged anal canal is a devastating complication of a pull-through procedure for Hirschsprung disease, which can result in permanent fecal incontinence [
      • Bischoff A
      • Frischer J
      • Knod JL
      • Dickie B
      • Levitt MA
      • Holder M
      • et al.
      Damaged anal canal as a cause of fecal incontinence after surgical repair for Hirschsprung disease - a preventable and under-reported complication.
      ]. These complications seem to occur more frequently than most clinicians acknowledge [
      • Levitt MA
      • Dickie B
      • Peña A.
      The Hirschsprungs patient who is soiling after what was considered a “successful” pull-through.
      ]. Sphincters are required for tone around the pull-through (internal sphincter) and to voluntarily close the neo rectum to hold stool, to be released at the appropriate time (external sphincter). The anal canal is necessary for sensation, which allows detecting of the differences between solid, liquid, and gas, and for proprioception to detect stretch of the neorectum. Injury to the anal sphincters and loss of the dentate line are two iatrogenic causes for true fecal incontinence following a transanal pull-through in Hirschsprung's disease beside overflow soiling and soiling related to a fast moving colon (hypermotility) [
      • Langer JC
      • Rollins MD
      • Levitt M
      • Gosain A
      • de la Torre L
      • Kapur RP
      • et al.
      Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease.
      ]. Intact but non-relaxing sphincters can also lead to soiling and this is well treated with botulinum toxin injection of the anal canal [
      • Halleran DR
      • Lu PL
      • Ahmad H
      • Paradiso MM
      • Lehmkuhl H
      • Akers A
      • 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.
      ].
      Fecal incontinence due to sphincter damage has no optimal treatment. Most of the published techniques involve adult patients with incontinence results from obstetric trauma after vaginal delivery or anorectal surgery (prostate cancer, haemorrhoids, perianal sepsis, rectal cancer) [
      • Kuller JA
      • Wells SR
      • Thorp Jr, JM
      • Bowes Jr., WA
      Obstetric damage and faecal incontinence.
      ,
      • Muñoz-Yagüe T
      • Solís-Muñoz P
      • Ciriza de los Ríos C
      • Muñoz-Garrido F
      • Vara J
      • Solís-Herruzo JA
      Fecal incontinence in men: causes and clinical and manometric features.
      ,
      • Lunniss PJ
      • Gladman MA
      • Hetzer FH
      • Williams NS
      • Scott SM.
      Risk factors in acquired faecal incontinence.
      ,
      Cook, Cook, Mortensen
      Management of faecal incontinence following obstetric injury.
      ,
      • Kim T
      • Chae G
      • Chung SS
      • Sands DR
      • Speranza JR
      • Weiss EG
      • et al.
      Faecal incontinence in male patients.
      ]. A sphincter reinforcement for adult patients with rectal prolapse was introduced and performed by Thiersch in 1891 using a silver wire encircling the anus [

      Carrasco AB. Contribution a l'etude du Prolapsus du Rectum. Verlag nicht ermittelbar, 1935.

      ,
      • Bérenger-Féraud
      • Bérenger-Féraud
      Contribution à l’étude des vestiges des pratiques religieuses de l'antiquité chez les Provençaux de nos jours: L'immersion de la statue du saint.
      ]. Recent modifications of this technique by using elastic bands in adults with fecal incontinence has shown promising results [
      • Lim CH
      • Kang WH
      • Lee YC
      • Ko YT
      • Yoo BE
      • Yang HK.
      Standardized method of the thiersch operation for the treatment of fecal incontinence.
      ]. Other new techniques like autologous expanded mesenchymal stem cells infusions still lack demonstrated efficacy [
      • de la Portilla F
      • Guerrero JL
      • Maestre MV
      • Leyva L
      • Mera S
      • García-Olmo D
      • et al.
      Treatment of fecal incontinence with autologous expanded mesenchymal stem cells: results of a pilot Study.
      ].
      In children, incontinence is mostly treated with a medical management involving either laxatives for constipation or anti-motility agents for a fast-moving colon but in a patient with sphincter and/or anal dysfunction, these methods will not be successful. Rectal (retrograde), or antegrade enemas as part of a bowel management program are somewhat effective, although in Hirschsprung disease the results are suboptimal [
      • Sood S
      • Lim R
      • Collins L
      • Trajanovska M
      • Hutson JM
      • Teague WJ
      • et al.
      The long-term quality of life outcomes in adolescents with Hirschsprung disease.
      ]. If these prove successful, then patients with true fecal incontinence due to damaged or overstretched sphincters are then dependent for life on a mechanical program (enemas) to achieve social continence [
      • Kilpatrick JA
      • Zobell S
      • Leeflang EJ
      • Cao D
      • Mammen L
      • Rollins MD.
      Intermediate and long-term outcomes of a bowel management program for children with severe constipation or fecal incontinence.
      ]. Alternative therapies like sacral neuromodulation (SNM) or transcutaneous nerve stimulation (SNS) have shown some potential in improving urinary and fecal incontinence symptoms, by what is thought to be an enhancement of the sphincter function, but still need further investigations [
      • Dos Santos J
      • Marcon E
      • Pokarowski M
      • Vali R
      • Raveendran L
      • O'Kelly F
      • et al.
      Assessment of needs in children suffering from refractory non-neurogenic urinary and fecal incontinence and their caregivers’ needs and attitudes toward alternative therapies (SNM, TENS).
      ,
      • Thaha MA
      • Abukar AA
      • Thin NN
      • Ramsanahie A
      • Knowles CH.
      Sacral nerve stimulation for faecal incontinence and constipation in adults.
      ]. Nevertheless, this modality might be beneficial in combination with our described technique.
      In our first patient, the patulousness of the sphincters which led to the prolapsed pull-through was likely caused by overstretching of the sphincters during the original pull-through. In addition, there was no evidence of a dentate line, which can be explained by a transanal dissection that was started too low during the original procedure. With our idea to tighten the sphincter after excision of the prolapse in this patient, we are hopeful that the child will ultimately achieve voluntary bowel control. Thus far, even at his young age it appears that he is already achieving clinical improvements. The second patient is fully continent after this procedure, and thus we feel confident to offer this procedure to other patients with a similar anatomic concern.

      Conclusion

      The sphincter tightening procedure could be a beneficial maneuver for patients suffering from true fecal incontinence following a pull-through procedure for Hirschsprung disease.

      Author disclosure statements

      All authors have no disclosures or conflicts of interest.

      Level of evidence

      Level IV.

      Funding

      No funding received.

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