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.
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
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.
- Hirschsprung H. Stuhltragheit Neugeborener infolge von Dilatation und Hypertrophie des Colons. Jahrb Kinderheilkd 27:1-42, 1887.
- 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.
- Coran AG, Teitelbaum DH. Recent advances in the management of Hirschsprung’s disease. Am J Surg 2000 180(5):382-387.
- Soave F. Hirschsprung’s disease: Technique and results of Soave’s operation. Br J Surg. 1966 Dec;53(12):1023-7.
- Duhamel B. A new operation for the treatment of Hirschsprung’s disease. Arch Dis Child 1960;35: 38-39.
- Boley SJ. New modification of the surgical treatment of Hirschsprung’s disease. Surgery. 1964 Nov;56:1015-7.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- Gauderer MWL. Stomas of the Small and Large Intestine. In Pediatric Surgery, Fifth Edition, Eds. ONeill JA et al, Mosby. 1998, pp1349-1359.
- 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.
- 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.
- 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.
- 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.
Requests for reprints should be sent to:
International Pediatric Endosurgery Group (IPEG)
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