Guidelines for Surgical Treatment of Hirschsprung’s Disease

Introduction

Hirschsprung’s disease (HD), or colonic aganglionosis, was first described clinically by Harald Hirschsprung and later precisely defined by Ovar Swenson. Surgical therapy has been the cornerstone of management for the correction of colonic aganglionosis since Swenson described the first successful pull-through operation in 1948.

The management of aganglionosis has evolved significantly over the past 60 years. Technical approaches different from Swenson’s original pull-through procedure have been developed and further modified., Surgical treatment of HD initially involved a three-stage sequence with colostomy creation, then the pull-through procedure with creation of a temporary “protecting” colostomy, followed by eventual colostomy closure.

Many pediatric surgeons currently perform a two-stage procedure with initial colostomy and subsequent pull-through only, eliminating the need for a final colostomy closure. Primary pull-through in the newborn and those older infants and children with delay in diagnosis has been advocated by some for several years.7–9 There has been a gradual accumulation of data that has suggested that pull-through can be performed selectively in the newborn as a primary procedure without a colostomy. However, concern for accurate diagnosis and the performance of such a technically demanding operation without diversion mandates expertise in proceeding with this approach.

The development of minimally invasive techniques, experience, and instruments for babies has allowed the application of this approach for the management of HD in selected newborns and older children.10-13 First reported in the mid-1990’s, these minimally-invasive techniques have recently been adopted and modified by pediatric surgeons throughout the world.

Definition

Hirschsprung’s disease is a rare congenital disease of the intestine, characterized by the absence of autonomic ganglion cells in the wall of the colon. It affects roughly 1 in 5000 live births. The cause is unknown. However, it is associated with genetic abnormalities anomalies, including Trisomy 21 (Down syndrome).

Diagnosis

Delay in passage of meconium, obstipation, abdominal distention, bilious vomiting, and failure to thrive may be symptoms of HD. A precise diagnosis is obtained by rectal biopsy, interpreted by an experienced pediatric (or gastrointestinal) pathologist. Contrast radiography is useful in the diagnosis and in estimating the extent of the disease in many patients, but may not be helpful in this regard in newborns. Once the diagnosis is established, treatment is aimed at preparing the patient for surgical correction.

Preoperative Management

Concomitant with measures to secure a diagnosis, the patient is decompressed with a nasogastric (or orogastric) tube and rectal irrigations. This has been felt to decrease the risk of preoperative Hirschsprung’s enterocolitis. Associated anomalies are investigated. Any fluid or electrolyte imbalances should be corrected. Severe malnutrition is unusual but, if identified, should be treated. Total parenteral nutrition may be required.

If successful intestinal decompression has been accomplished, primary pull through may be undertaken in some patients. Some pediatric surgeons have had success in managing patients with HD with home rectal irrigations for weeks or even months prior to definitive surgery. This has been felt, in some cases, to be preferred since it allows small babies the chance to increase in size and weight prior to surgery.

If a staged approach is planned, colostomy should be performed as soon as the diagnosis is confirmed by rectal biopsy. Immediate operation should be undertaken if irrigations are ineffective. Persistent abdominal distention that cannot be decompressed is a deterrent to laparoscopic pull-through. The occurrence of definite enterocolitis preoperatively is usually considered a contraindication for primary pull-through, and a leveling colostomy should be performed. The presence of other associated anomalies may dictate a staged approach. Broad-spectrum antibiotics are given prophylactically.

The choice of the operative approach depends on identifying the distal-most segment of bowel with ganglion cells. Frequently this can be suggested by contrast enema, but is typically defined by biopsy with frozen section examination accomplished in the operating room. The diagnosis of the transition zone should be secured by histologic examination before proceeding with the colostomy or definitive pull-through.

A small percentage of HD patients have so-called “ultra short segment” HD with the transition zone close to the anus. However, approximately 75-80% of patients with HD have the transition zone in the rectosigmoid area. The remainder of cases involves longer segments of aganglionic colon, rendering the pull-through more challenging and the potential outcome not as favorable.14 Very few cases of laparoscopic pull-through for long segment disease have been reported to date.15

Surgical Techniques

General Principles

Once the transition zone is identified, standard techniques for colostomy are used for diversion in cases where a two-stage procedure is performed.16

Definitive correction typically utilizes one of the 3 fundamental operations for pull-through. The techniques of these procedures are well documented.17 Any can be used as a technique for primary or a staged repair using laparoscopic approach. Final frozen section biopsy of the pull-through segment prior to anastomosis is recommended. Some recent reports suggest that primary transanal pull-through can be done for patients having a very low transition zone.18-20 The surgeon should be confident that the transition zone is low enough, preferably below the upper sigmoid, before beginning this type of operation. Some authors advocate a laparoscopic biopsy as the first step before beginning the transanal approach.12, 15

Laparoscopic Operations

A pediatric surgeon who desires to treat HD with minimally invasive surgery should meet the qualifications that allow him/her to practice pediatric surgery in his/her country and should be considered competent by peer review based on ample experience, including either residency training or formal laparoscopic course(s).

Specific training in advanced pediatric laparoscopic techniques, either in fellowship or through special postgraduate courses, is recommended. The surgeon should perform laparoscopy regularly in practice and have experience in basic laparoscopic procedures such as cholecystectomy, appendectomy and fundoplication. An experienced assistant is helpful.

Georgeson et al, representing six pediatric surgical centers, summarized the effectiveness of the one stage laparoscopic approach for HD utilizing a Soave technique in eighty patients19. Lacking long term follow up for continence, they have observed no major morbidity and significantly shorter days of hospitalization than reported standards for the two or three stage procedures.

Before embarking on primary pull-through, the transition zone should be identified with a frozen section biopsy. Discovery of long segment disease may be a deterrent to primary pull-through although not an absolute contraindication. Experience using this technique for long segment disease is described, but there are no series yet reported that have described outcomes data.

The intrabdominal portion of the laparoscopic technique is not different from that of open primary pull-through. Three ports are usually used in the upper abdomen using standard insertion techniques. A right lower quadrant port can be used for colon manipulation, especially when performing the Duhamel or Swenson procedures. Initial dissection consists of mobilizing the sigmoid colon down to and opening the peritoneal reflection. Bipolar cautery or ultrasonic dissection is optimal to minimize the risk of collateral damage. Further dissection into the pelvis can be done, as in the Swenson or Duhamel approach, but is not necessary for the Soave endorectal approach. Great care should be taken to avoid collateral structures, especially the left ureter and the vas deferens. In the Soave technique, the mucosal proctectomy is usually done from the rectum upward, not from the top down as traditionally described. It is helpful if the surgeon has experience using this technique for patients with ulcerative colitis and familial polyposis.

Summary

The implementation of laparoscopy for the primary pull-through operation has altered the management of rectosigmoid aganglionosis by allowing the surgeon to safely use an established concept (pull-through) while eliminating a major source of morbidity (staged correction and laparotomy). The distinct advantage of primary pull-through is never having a colostomy, with its consequent morbidity. There is rapid recovery and less postoperative perianal excoriation. Also, the laparoscopic technique avoids significant external and intrabdominal scarring.

The critical factors in the success of this approach include the experience of the surgeon and that of the pathologist. While the long-term outcomes of this approach regarding continence and constipation are not established, preliminary data suggest, at least, similar results to the open techniques. It is becoming the preferred approach of choice over the open and staged techniques, particularly for the healthy patient with rectosigmoid disease.

References

  1. Hirschsprung H. Stuhltragheit Neugeborener infolge von Dilatation und Hypertrophie des Colons. Jahrb Kinderheilkd 27:1-42, 1887.
  2. Swenson O, Bill AH. Resection of the rectum and rectosigmoid with preservation of sphincter for benign spastic lesions producing megacolon. Surgery 24:212-220, 1948.
  3. Coran AG, Teitelbaum DH. Recent advances in the management of Hirschsprung's disease. Am J Surg 2000 180(5):382-387.
  4. Soave F. Hirschsprung’s disease: Technique and results of Soave’s operation. Br J Surg. 1966 Dec;53(12):1023-7.
  5. Duhamel B. A new operation for the treatment of Hirschsprung’s disease. Arch Dis Child 1960;35: 38-39.
  6. Boley SJ. New modification of the surgical treatment of Hirschsprung's disease. Surgery. 1964 Nov;56:1015-7.
  7. So HB, Becker JM, Schwartz DL, Kutin ND. Eighteen years’ experience with neonatal Hirschsprung’s disease treated by endorectal pull-through without colostomy. J Pediatr Surg 33(5):673-675, 1998.
  8. Carcassonne M, Morrison-Lacombe G, Letourneau JN. Primary corrective operation without decompression in infants less than three months of age with Hirschsprung’s disease. J Pediatr Surg. 1982 Jun;17(3): 241-243.
  9. Georgeson KE, Cohen RD, Hebra A, Jona JZ, Powell DM, Rothenberg SS, Tagge EP. Primary laparoscopic-assisted endorectal colon pull-through for Hirschsprung's disease: a new gold standard. Ann Surg 1999 May; 229(5):678-82; discussion 682-3.
  10. de Lagausie P, Berrebi D, Geib G, Sebag G, Aigrain Y. Laparoscopic Duhamel procedure. Management of 30 cases. Surg Endosc 1999 Oct;13(10):972-4.
  11. Curran TJ, Raffensperger JG. Laparoscopic Swenson pull-through: a comparison with the open procedure. J Pediatr Surg. 1996 Aug;31(8):1155-6; discussion 1156-7.
  12. Georgeson KE, Fuenfer MM, Hardin WD. Primary laparoscopic pull-through for Hirschsprung's disease in infants and children. J Pediatr Surg 1995 Jul;30(7):1017-21; discussion 1021-2.
  13. Rothenberg SS, Chang JH. Laparoscopic pull-through procedures using the harmonic scalpel in infants and children with Hirschsprung's disease. J Pediatr Surg. 1997 Jun;32(6):894-6.
  14. Endo M, Watanabe K, Fuchimoto Y, Ikawa H, Yokoyama J. Long-term results of surgical treatment in infants with total colonic aganglionosis. J Pediatr Surg. 1994 Oct;29(10):1310-1314.
  15. Georgeson KE. Laparoscopic pull-through for Hirschsprung’s Disease. In Hirschsprung’s Disease and Allied Disorders, Second Edition, Eds. Holschneider AM and Puri P. Harwood Academic Publishers. 2000, pp 301-310.
  16. Gauderer MWL. Stomas of the Small and Large Intestine. In Pediatric Surgery, Fifth Edition, Eds. ONeill JA et al, Mosby. 1998, pp1349-1359.
  17. DH Teitelbaum et al. Hirschsprung's Disease and Related Neuromuscular Disorders of the Intestine. In Pediatric Surgery, Fifth Edition, Eds ONeill JA et al, Mosby. 1998, pp 1381-1424.
  18. Langer JC, Seifert M, Minkes RK. One-stage Soave pull-through for Hirschsprung's disease: a comparison of the transanal and open approaches. J Pediatr Surg 2000 Jun;35(6):820-2.19.
  19. Langer JC, Durrant AC, de la Torre L, Teitelbaum DH, Minkes RK, Caty MG, Wildhaber BE, Ortega SJ, Hirose S, Albanese CT. One-stage transanal Soave pullthrough for Hirschsprung disease, A multicenter experience with 141 Children. Ann Surg 2003 Oct;76,2003.
  20. Dela Torre L, Ortega J. Transanal endorectal pullthrough for Hirschsprungs disease. J Pediatr Surg 33: 1283-1286, 1998.
This guideline was prepared by the IPEG Guidelines Committee and was reviewed and approved by the Executive Committee of the International Pediatric Endosurgery Group (IPEG) July, 2004.

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