Pediatric Surgery Service, University Clinical Hospital Virgen de la Arrixaca, Murcia, Spain.

 

 

One specific complication of Hirschsprung disease’s surgery is obstruction, that can arise from mechanical causes, such as narrowing of the residual cuff. It is a well known complication, although few reports are at respect. 

 

Key words:

laparoscopy, hirschsprung disease, muscular cuff, constipation.

 

 

A review article was done, serching articles in pubmed database, using the words „laparoscopy“, „hirschsprung disease“, „muscular cuff“ and „constipation“. The most relevant articles was selected, according to the year of publication, recognized names of authors, and the impact factor of the journal.

 

 

Hirschsprung disease (HD) was described by Harald Hirschsprung in 1887 and occurs approximately once in every 5000 live-born infants(1). Since the introduction of operative treatment of HD by Swenson in 1948(2-3), methods gradually evolved from the original 3-stage procedure to a 1-stage procedure(2,4).  Endorectal pull-through for HD was described in 1964 by Soave(5). The 1-stage transanal endorectal pull-through (TEPT) operation was introduced in 1998 by De La Torre(6).
 

A significant number of children whom undergo a successful pull-through will develop postoperative complications(7). The most common and serious long-term complications after definitive treatment for  HD can be divided into 3 groups: soiling/incontinence, persistent problems with the passage of stool (eg, constipation), and recurrent Hirschsprung associated enterocolitis(7). Recent reports have recognized that obstructive symptoms are occurring in 9% - 40% of children after what appears to be technically excellent operation(7,8). Recurrent obstruction can arise from mechanical or functional causes. Narrowing of the residual cuff and coloanal anastomotic stenosis are listed as causes of the former(9,10).
 

In the original transanal pull-through procedure, a long rectal muscular cuff (5 – 7 cm) was dissected and left for anocolic anastomosis, which would sometimes lead to postoperative obstructive symptoms and enterocolitis even through dividing the cuff in the posterior wall(11).
 

As we can see, obstructive symptoms due to the cuff is a well known complication, but only a few reports exist about the treatment of this complication, especially when it comes to a laparoscopic approach to relief the muscular cuff. Here it was done a review article in pubmed database, using the words „laparoscopy“, „hirschsprung disease“, „muscular cuff“ and „constipation“, to reveal how many reports exist about this procedure.
 

 

Muscular cuff after a Soave or TEPT procedure, can result in a mechanical obstruction if not adequately split or if a significant length of cuff was left behind(11-12). Many experts recommend a posterior muscular cuff´s myectomy fully down to the dentade line to prevent residual constipation(9-10, 13). Nevertheless, Levitt, De la Torre and Nasr said, that even though the cuff may have been split during the original operation, sometimes it can scar down or roll up and cause obstruction(12, 14-15). The residual muscular cuff can cause a constricting fibrotic ring in the pelvis resulting in a functional stricture(12).  Many of these children have postoperative symptoms that are identical to their symptoms at initial presentation(1).
 

A problematic rectal muscular cuff can be palpable on rectal examination. It can also be suspected on contrast enema with an increased distance between the pull-through segment and the sacrum(9,12). Sometimes, a MRI is also needed to confirm the diagnosis.  
 

The initial descriptions of the TEPT involved a long mucosal dissection leaving a rectal muscular cuff that extended above the peritoneal reflection(15). The trend now is to leave a few centimeter rectal muscular cuff or no cuff at all, which will hopefully reduce the cuff problems observed, as the avoidance of rolling down and forming a constricting ring around the pull-through bowel, with a lower risk of obstruction and enterocolitis(12,14,16). A short cuff could be an important factor in reducing severe rectal stenosis and enterocolitis(17). Nasr and Langer also noticed that the incidence of enterocolitis (9% vs. 30%; p=0.1) and rectal stenosis requiring daily dilatation (5% vs. 30%; p=0.047) decreased in the short cuff group in comparison with the long cuff group, as well as decreasing in hospital stay (1.9 ± 0.6 vs. 2.7 ± 0.9; p=0.001)(15,17).
 

The disadvantage of a transanal Swenson, leaving no muscular cuff,  is that dissection on the outside of the rectum deep in the pelvis may increase the risk of injury to pelvic nerves and vessels, and to the prostate, urethra, or vagina if dissection is not close to the rectal wall(14,18). To avoid these complications, in the beginner´s hands is best done by continuing mucosectomy for a relatively long length. Once the technique of mucosectomy is mastered, a very short rectal cuff (1 – 2 cm) can be left(18).  
 

In a 12-question survey done to the members of American Pediatric Surgical Association at 2009, the median length of cuff overall for those who leave a cuff was 2.4 cm and a divided muscular cuff is reported by 55%(19). It is difficult to find the ideal balance because the incidence of postoperative enterocolitis or constipation does not reach statistical significance when outcomes of long and short cuff lengths are compared(18). Further evaluation is required to determine whether application of the short muscular cuff can change the postoperative stabilization period(2).
 

If a redo Soave procedure has to be done, it is not advisable to do another submucosal dissection of the already previously pulled through colon, to avoid leaving behind 2 seromuscular cuffs(20). In a Peña´s article which reviewed 51 patients with complications that required a reoperation, 21 patients had some kind of stricture or acquired atresia of the rectum(21).  He postulated a posterior sagital approach to be used in the reoperation of these patients, as it has also done by Kimura, in 1993(22). The area of the stricture is usually located in a rather high area of the pelvis to be approached transanally and rather low to be approached abdominaly(21).  Although this usefull recomendation, we have to think that maybe a laparoscopic approach is the best option because it is less invasive than a sagital approach, without making any incision, no stitches, without touching the pulled through colon, and no increasing the risk of a rectocutaneous fistula. The use of laparoscopy has the advantage of initial confirmation of the diagnosis and proper visualization of the rectal cuff, which can be incised or excised either anteriorly or posteriorly(9).

Only two articles about laparoscopic procedure have been find. One case report published in 2004(23), of a patient who developed early postoperative severe constipation after TEPT due to unusual folding of the muscular cuff rim, which tightly narrowed the pulled-through colon. The complication was diagnosed and treated by laparoscopy. And another case report in 2012(9), where two cases are described about two child having obstructive symptoms due to a tight muscular cuff, that could be released with a laparoscopic approch without any incidence.

 

 

There are many complications reported about the muscular cuff, but only a few reports on the release by a laparoscopic approch. A thorough split of the muscular cuff, and perhaps leaving a shorter one, has to be consider in the initial surgery. The obstructive complication could be managed safely by a laparoscopic cuff myectomy.

References

 

1. Dasgupta R, Langer JC. Evaluation and management of persistent problems after surgery for Hirschsprung disease in a child. J Pediatr Gastroenterol Nutr. 2008;46(1):13-9. 

2. Kim HY, Oh JT. Stabilization period after 1-stage transanal endorectal pull-through operation for Hirschsprung disease. J Pediatr Surg. 2009;44(9):1799-804. 

3. Masiakos PT, Ein SH. The history of Hirschsprung´s disease: then and now. Semin Colon Rectal Surg. 2006;17:10-19. 

4. Wester T, Rintala RJ, Langen J, Wester T, Hadidi A, Bagolan A, et al. Early Outcome of Transanal Endorectal Pull-Through with a Short Muscle Cuff during the Neonatal Period. J Pediatr Surg. 2004;39(2):157-60. 

5. Soave F. Hirschsprung´s disease: a new surgical technique. Arch Dis Child. 1964;39:116-124. 

6. De la Torre-Mondragon L. OSJ. Transanal Endorectal Pull-Through. J Pediatr Surg. 1998;33(8):1283-6. 

7. Ralls MW, Coran AG, Teitelbaum DH. Reoperative surgery for Hirschsprung disease. Semin Pediatr Surg. 2012;21(4):354-63.

8. Langer JC. Persistent obstructive symptoms after surgery for Hirschsprung’s disease: Development of a diagnostic and therapeutic algorithm. J Pediatr Surg. 2004;39(10):1458-62. 

9. Hay SA, Shafei I El, Debeky M El, Bassiouny A. Release of obstructing rectal cuff following transanal endorectal pullthrough for Hirschsprung’s disease: A laparoscopic approach. Ann Pediatr Surg. 2012;8(3):90-2. 

10. Shimotakahara A, Yamataka A, Kobayashi H, Miyano GO, Kusafuka J, Lane GJ, et al. Transanal Endorectal Pull-Through for Hirschsprung ’ s Disease. 2006;16(5):540-2. 

11. Yang L, Tang ST, Cao GQ, Yang Y, Li S, Li SW, et al. Transanal endorectal pull-through for Hirschsprung’s disease using long cuff dissection and short V-shaped partially resected cuff anastomosis: Early and late outcomes. Pediatr Surg Int. 2012;28(5):515-21. 

12. Levitt MA, Dickie B, Peña A. Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure. Semin Pediatr Surg. 2010;19(2):146-53.

13. Kubota A, Kawahara H, Okuyama H, Oue T, Tazuke Y, Okada A. Clinical outcome of laparoscopically assisted endorectal pull-through in Hirschsprung’s disease: Comparison of abdominal and perineal approaches. J Pediatr Surg. 2004;39(12):1835-7. 

14. De La Torre L, Langer JC. Transanal endorectal pull-through for Hirschsprung disease: technique, controversies, pearls, pitfalls, and an organized approach to the management of postoperative obstructive symptoms. Semin Pediatr Surg. 2010;19(2):96-106.

15. Nasr A, Langer JC. Evolution of the technique in the transanal pull-through for Hirschsprung’s disease: effect on outcome. J Pediatr Surg. 2007;42(1):36-40. 

16. Rintala RJ. Transanal coloanal pull-through with a short muscular cuff for classic Hirschsprung’s disease. Eur J Pediatr Surg. 2003;13(3):181-6. 

17. Liem NT, Hau BD, Quynh TA, Anh VTH. Early and late outcomes of primary laparoscopic endorectal colon pull-through leaving a short rectal seromuscular sleeve for Hirschsprung disease. J Pediatr Surg. 2009;44(11):2153-5.

18. Pratap A, Gupta DK, Shakya VC, Adhikary S, Tiwari A, Shrestha P, et al. Analysis of problems, complications, avoidance and management with transanal pull-through for Hirschsprung disease. J Pediatr Surg. 2007;42(11):1869-76. 

19. Keckler SJ, Yang JC, Fraser JD, Aguayo P, Ostlie DJ, Holcomb GW, et al. Contemporary practice patterns in the surgical management of Hirschsprung’s disease. J Pediatr Surg. 2009;44(6):1257-60.

20. Hassan SH, Hashish AA, Fayad H, Elian A, Elatar A, Afify M, et al. Redo Surgery for Hirschsprung’s Disease. 2008;4(1):42-50. 

21. Peña A, Elicevik M, Levitt MA. Reoperations in Hirschsprung disease. J Pediatr Surg. 2007;42(6):1008-14. 

22. Kimura K, Inomata Y, Soper RT. Posterior sagittal rectal myectomy for persistent rectal achalasia after the Soave procedure for Hirschsprung’s disease. J Pediatr Surg. 1993;28(9):1200-1. 

23. Falchetti D, Dessant A, Villanacci V, Iannuccelli M. Laparoscopic relief of obstructing folded muscular cuff after transanal pull-through for aganglionosis. Surg Endosc. 2004;18(4):717-8.